CANCER SEMINAR
SECOND SERIES. VOLUME ONE MAY, 1979 NUMBER THREE Juan A. del Regato, Editor
CONTENTS ATYPICAL WILMS' TUMOR J. F. Wiot, M.D. and R. Clark, M.D...... 1· 4 TRANSITIONAL CELL CARCINOMA OF THE RENAL PELVIS R. McLelland, M.D...... 4-10 TRANSITIONAL CELL CARCINOMA OF THE KIDNEY PELVIS M. W. Elson, M.D ...... 10-13 WILMS' TUMOR IN AN ADI:JLT Harold G. Jacobson, M.D ...... 13·16 .. j WILMS' TUMOR IN AN ADULT •• R. Isbell, M.D. and Manuel A. Carta, M.D ...... , .... 16·18 ANG!OMYOLIPOMA OF THE KIDNEY H. Ramirez, .M.D .. H. Cruz·Ortiz, M.D. and J. Ceballos Labat, M.D ...... 18-22 WILMS' TUMOR WITH NEUROGENOUS DIFFERENTIATION Yvon LeGal, M.D ...... , ...... 22·25 PLEOMORPHIC LIPOSARCOMA OF THE KIDNEY D. Eisert, M.D. and A. A. Effron, M.D ...... • ...... 25·28 RETROPERITONEAL FIBROSIS A. F. Lalli, M.D. and W. A. Hawk, M.D...... 28·31 MALIGNANT LYMPHOMA OF RETROPERITONEAL SPACE F. I. Andres, M.D., H. J. Conlan, 11'I.D., M. Elson, M.D. and J . F. Wilson, M.D ...... 31·34 CONGENITAL MESOBLASTIC NEPHROMA J. Durham, M.D. and D. Meyers, M.D ...... 34·39 CARCINOSARCOMA (?) OF THE BLADDER Joseph Kuzma, M.D...... 39·43 TRANSITIONAL CELl. CARCINOMA OF THE BLADDER Paul Berger, M.D...... 43·46 LEIOMYOSARCOMA OF THE BLADDER J.D. Cox, M.D. and H. J. Conlan, M.D ...... 46-48 MUCINOUS ADENOCARCINOMA OF' 'l'HE BLADDER Carlos Perez·Mesa, M.D...... 48·50
EDITORIAL CONSULTANTS Tumor Radiodiagnosis...... Arthur Graham, M.D., Tampa, FL Tumor Surgery...... Juan Bolivar, M.D .. Tampa, FL Tumor Histopathology...... , , ...... Henry Azar, M.D .. Tampa, FL EDITORIAL OFFICE VETERANS ADMINISTUATION MEDICAL CENTER Department of Radiology 13000 North 30th Street, Tampa, Florida 33612
This second series of CANCER SEMINAR is printed by Joe B. Klay and Sons. Inc. in Tampa, Florida. J. A. dl)l Rcgato. M.D .. editor. Subscription rat6 $10.00 annually. Address. The second series of CANCER SEMINAR's has established itself under the auspices of the University of South Florida and the Veterans Administration Medical Center of Tampa. The third CANCER SEMINAR of the new series was devoted t·o the diagnosis and treatment of tumors of the urinar.y tract. Dr. Milton f~lkin, eminently qualified diagnostic radiologist, is part.icularly gifted in this area. The reader will ·en:joy the fine considerations that complement the radiographic examinations, the master's perception thattnake Dr. Elkin one· of the best teachers in his specialty. Dr. Fathollah Mostofi displayed his great ex· pericnce and his acknowledged refined judgment of histopathology of urinary tumors: we are additionally indebted to Dr. Moswfi for choosing the ap· propriate photomicrographs for this publication. The reader will be impressed by the extemporaneous discussions of each problem by Dr. Willet F. Whitmore, Jr.: a true . surgical oncologist, he evaluates the diagnostic possibilities and bas.es. his therapeutic opinions on multidisCiplinary experiences, rather than on mere surgical technology or ability. Dr. Whitmore's didactic discussions constitute and important contribution to this CANCER SEMINAR. These educational exercises require the cooperation of a number of friends who provide us with the interesting··cases and numerous dedicated workers who labor for ,months before the presentations and discussions: to all we re· main infinitely grateful. The help and support that we receive !rom the Department of Pathology ac count for a great deal of the quality of these proceedings. Dr. Henry Azar is in himself an assurance of the quality in the selection · of cases. Mr. Walter McAllister, a .dedicated craftsman, is· responsible for the fine quality of our slides. This publication is primarily due to the enthusiasm and support of the Department of Radiology and its. chairman, Dr. Arthur D. Graham: Dr. Graham's associates and staff all contribute in no littl.e measure to the organization and success of the CANCER SEMINAR. lt is our privilege to be able to continue these educational exercises in Florida, and we feel grateful. J. A. del Regato, M.D. Tampa, Florida May, 1979 MlLTON ELKIN, M.D. Professor of Radiology, Albert Ein· stein College of Medicine, New York. Dr. Elkin is one of the finest didacti· tians in American radiodiagnosis. F. K. MOSTOFI, M. D. Chief. Genitourinat·y P<>thology De· partment, Armed Forces lntitute of Pathology, Was hington, D.C. Dr. Mostofi is a respected world authority in histopathology of the urinary tract. WILLET F. WHITMORE, ,JR.. M.D, Professor of Surgery (Urology). Cornell University Medical College. New York. Dr. Whitmore is a perfect example of the.surgical oncologist in his specialty. l. ATYPICAL WILMS' TUMOR Contributed by J. F. Wiot. M.D. and U. Clark, M.D .. Cincinnati, Ohio The patient was a 14-year-old girl when she was phritis, which can ))roduce a sizable mass, and the admi~ted in March 1974 because of hematuria of lesion can be hypervascular. However, the four weeks duration and a long history of calcification often consists of calculi or possibly enuresis. On physical examination, a tender mass slight calcification in the inflammatory mass, not was felt below the right costal margin. The blood the massiv e calcification. seen her.e. r would con count was normal, and there were no urine abnor sider this diagnosis unlikely. 3) Angiomyolipoma malities; se1·um creatinine was 0.9 mgm!IOO mi. would be unusual in this age unless it were part ot the tuberous sclerosis complex, a .. d then I would Dr. Elkin: Roentgenograms show a large mass expect multiple and bilateral discr:ete vascular le· of the lower pole of the right kidney. extending sions. Calcification does occur in angiomyoli poma, an~c r iorly and well below the expected lower though n o~ commonly, and in my experience iLhas border of the kidney to at least the level of the been curvilinear calci£ication and slight in crest of the ilium; the upper pole calyces are amount. 1) Wilms' tumor can infrequently occur in opacified, th01Jgh they are distorted by pressure this age group (almost all are diagnosed before from the mass. The left kidney and the visualized age 12). Usually. the vessels arc not as wild as bones appear normaL The striking feature is the they are in the angiogram of this patient. • ~ massive collections of mottled calcifications in the However. ca.lcification is not often seen mass. Unfortunately, detail is not good enough to radiologitally in· Wilms' tumor. 5) Primary determine whether or not there is ossification in osteogenic sarcoma of the kidney is a rare tumor. addition to the calcification. A single film of the only a handful having been reported; the few selective angiography shows abnormal vessels in reported primary osteogenic sttrcomas of the the mass extending beyond the upper pole of the kidney occurred in an older age group, e.g. 50 to kidney; the vessels are abnormal in course, ir 80 year age group. and ~ hus, this would be a very regular in caliber, and in some areas there is the unlikely diagnosis [or t his· case. I would consider suggestion of puddling of contrast medium. I this possibility more comfortably if 1 could see believe that the angiographic findings are those evidence of ossification in the mass. 6) Metastases of a malignant neoplasm. Fi.g. 1-Large mas.s of 1he l•)wer r•oh: ur the right kidney 1,\'Hh mott.led <:Stl c:H i ~ations . say it is not a neuroblastoma. Angiomyolipoma would be a possibility except · that most of those we see are single lesions and are in middlc·agcd women. II we see them in young people, t hey usually are par t of a t uberous sclerosis complex. in which case they are apt to be mult iple lesions. Tbe cases of angiomyolipoma with calcification that I've seen have been very few, and in those cases, t he calcific.ation has been very slight: a few lines of calcification a lmost like a rim: I do not think this \vould · be an angiomyolipoma. Retroperitoneal tumor. yes- a kidney tumor is a retroperitoneal tumor. Dr. del Regato: Drs. Norman Rosenthal of Tampa and Manuel Viamonte of Miami suggested a diagnosis of Wilms' tumor. Dr. H. Esrov of Miami Beach, Florida offered a n impression of chondrosarcoma. Dr . N. W. E lson of Milwaukee proposed angiomyolipoma. Operative F'indings: On March 19, 1974 a right radical nephrectomy was carried out. A calcified tumor was found with a mixture of translucent areas and yellow-white portions as well as cystic formations. Fig. ;~-:;;clf!t' Li''l' angiography shuws a hnnrmal \'C:?scls, ir· Dr. Moslofi: T he n>eoplasm is densely cellular l'f.'J;UI:lr culiber and su !fge~ l i<~n of puddling. wit h a large area of chondroid and car t ilage for m~t tion; areas of necrosls and hyalinization are the diagnosis would be the history, i.e. does she have a bone tumor? also seen. In t he densely cellulttr areas. the cells are undifferentiated but fairly unifo r m, have If we saw this lesion in an adult. t he first round or oval nuclei, surrounded by a clear halo, diagnosis would be renal cell carcinoma. with fine chromatin pattern and rare, if any. Radiologically demonslrable calcification does oc nucleoli. Oecasionallv, more than one nucleus is cur in about 10 to 15% of these cancers, and seen. The cytoplasm "is scant, a nd the cell borders sometimes t he calcification is quite extensive. are poorly defined: mitotic figm·es are rare. T he However, what about renal cell carcinoma in a 14 cells occm· in sheets. occasionally forming cord or year girl? This would be unusual but not impossi t ubule-like structures (Fig.4). The stroma varies ble. Lynne and·Machiz reported diagnosis of renal from place to p lace. At t im.es it is scant; at other cell carcinoma in four children, ages 5 to 16 years times it is hyalinized. In some areas the tumor is old. It has been stated that renal cell carcinoma highly vascularized with thin-walled 'vascular makes up 2 to 5% of all malignant t umors in channels somet imes surrounded by tumor cells children. (t'ig.5). . The cartilaginous areas present a band-like ap Dr. Elkin's Impression: pearance at one marg·in and seem to merge with · 1) RENAL CELL CARCIN01\L-\ the cellular areas (Fig.6). The cells in t he chon- 2) WILMS' TUMOR 3) METASTATIC OSTEOSArtCmLI\ 1-'ig. .j-Cdl.s Jurming lubuh: ·likt:: stri c l urc~ (x:l5(1), Raaiologic impressions submitted: Renal ade n (tcar~: inorna ...... 25 Wilms' tumor ...... : . . . . . ~ ...... ~ .19 Sarcomas {c>SLc{)·. fibru·, chondro.-, lif)CJ-. angil)·) ...... : . . . : .... 14 N f! urQ blas~omR . . . • .. . ~ ...... 14 Angiomyolipom Fig. l-llighly ,·ascularlztd tumor with thin-walled ehann~ l ii the patient is a child. the preferred diagnosis is (x I!i O). atypical Wilms' with chondrosarcomatous areas. One must admit that if tho tumor occurred droid matrix vary somewhat in s ize, sh~pe and elsewhere than in the kidney and no renal in staining qualities. No distinct calcification or volvement was appar,ont, the tumor would he bone formation is present. Several areas of called mesenchymal chondrosarcoma. necrosis are seen, ranging from ischemic to hemorrhagic, in association with a thrombosed Dr. )!ostoli's Diagnosis: major blood vessel. A segment of the renal pelvis is avnilable, and there is a cystic space in the ATY PICAL WILMS' TUMOR substance or the tumor suggesting a calyx, but no Histopathologic diagnoses submitted: other renal elements arc identified. !'lephroblastoma (\Vilm•'J ...... 36 Malignant mesenchymiAI tum(lr ...... 13 'rhc majority of radiological diagnoses was un· Chondrosarcoma (ex.trMkeletal)...... 10 doubtedly arrived at because of the vascular pat· Mesoblastic n ephrnm:~ . , ...... 16 tem, which was considerably more prominent Hemangiopcl'icyt 2. TRANSITIONAL CELL CARCINOMA OF THE RENAL PELVIS Contributed by R. McLelland, M.D., Durham, North Carolina The patient w~s a 69·year·old woman in May found except for tenderness to palpation of the 1976 when she complained of left flank pain of left lower abdominal quadrant. The hemoglobin several months duration. polllikiilria and nyc· was 12 gm 0~; no abnorm~Jities were found in th~ turia. On examination no abnormalities were urine. 4 lion of the kidnev as well as absence of interlobar artery opacifica.tion in the lowe r half of the kidney. There are collections or abnormal vessels about the upper segment of the ureter. about the medial aspect of the lower pole of the kidney. and outside the medial aspec~ of the kidney just cephalad to the hilus. These all look like tumor vessels. apparently being fed by branches of pelvic arter ies. The previously noted mass at the lateral aspect of the kidney is not. seen now, but t hcrre is a paueily of vessels IlL Lhc lateral lower half of the kidney with deviation ()[ ves~els in this region and straightening of the border of the kidney f'hc. 1 - P'rl'!'o~Ur"· di.;turtion or ml'dia1 iUiJlCl"t or th UPP'-'r lo"'" of lht- n•nal p•·hi .... Or. Elkin: There is evidence of a previous myelogram. but I will pass this by as of no per· tinence to the present problem. I cannot be sure whether the irregularity of the upper 5 em of the ureter is due t.o intraluminal defects. I am thinking of a spreading type of mucosal lesion. s uch as transitional cell carcinoma or of an extr insic course. such as pressure from vessels or tumor around the ureter. One of tho lower calyces is opacified very fain tly. and thoro is tho suggestion of a filling defect in it. Thm·e is IH'essure distortion of the medial aspect of the up· per pttrt of the renal pelvis as by a mass in tho kidney and a roughly rounded 3.5 em zone of in· creased density in this r egion. I cannot be sure whether this represents merely a conrluence of shadows. such as Ouid in the stomach. or contrast medium from the renal calyces as may o<:cur with a necrotic neoplasm. There is also a mass in the lateral portion of the kidney, causing a bulge in the lateral renal border In selective an!l'iography. ther e is encascmcn~ of some of the smaller vessels at the lower por· Renal cell eart"inom:.l . .2'l Renal vein throm~l.!' . . 12 :\fet3.St3tk tumor .08 Renal tu~reuJ01is (}.; Otbtr .. 08 Or. Elkin: Renal vein thrombosis is a correct diagnosis. but of course, that is only part of the picture: I think it is renal vein thrombosis due to extension of tumor inLo the renal vein. Metastatic tumor is a reasonable diagnosis. I do not think renal tuberculosis would be a feasible diagnosis because of the clusters of vess•~ l s wo have around the kidney: r enal tuberculosis is basically a hypovascular lesion. Dr. del Regato: Drs. A. Conv ers of Miami and Paul Berger of Dunedin, Florida also offered an impression of transitional cell carcinoma of the kidney. Dr. H. Esrov of Miami Beach added that the transitional tell carcinoma probably had also invaded che renal vein. Dr. H. Ramirez·Jimenez of Mexico City offered renal carcin<>ma with renal vein thrombosis. Opera! ;,.e Findings: On June 10. 1976, a left nephrouretcreclomy and adrenalectomy were carried out. The lower half of the left kidney was Fi$t. 1-Scl ective phlebogram l'hU-A'~ hluck und rl lli n~: dcft•eh of n•n f'i,:. :.\-l'auth) ol \'('SS(-!;~ in lowtr h::tlr of thr kidm.") v.hlch up ~.n-c P>mRII. lerlobar ar teries of the lower half of the kidney'! Then the unusual vessels around the ureter and kidney might be peculiar collateral vessels. How ever, how would this explain Lhe phlebogr aphic findings, and more importantly. the retrograde pyelogram? Metastatic tumors to Lhe kidney llnd around t he upper segment of t ho ureter could invade the kidney, di~torting and a'm putating calyces, and even extending into veins . '!'his would be possible, I guess. and among metastases to the kidney I include the lymt1homa g•·oup. The diagnosis 1 favor is transitional cell carcinoma ol' the renal pelvis with extension into and even beyond the renal parenchyma. The ureteral abnormality might then also be due u l transitional cell carcinoma, either a multicentric focus or extension from the renal pelvis. Or. t:lkin's Impression: 1) TRANSITI01\'AL CELL CARCINOMA 2) MF.TASTATIC Tm!OR R:1diologic impressions submitted: 'J'ransition:tl cell cardnoma. . .37 squamous eells, but the predominant thing was transitional cell carcinoma. If we had not seen anything in the renal pelvis, either radi· ographically or in gross finding, our diagnosis would have been secondary carcinoma involving the kidney, However, the radiologic findings leave us no doubt that the primary tumor is a transitional cell carcinoma, deeply infiltrating renal parenchyma and with vascular invasion. Dr. del Regato: Drs. T. Dittmer of Cleveland and R. Komorowski of Milwaukee also made a diagnosis of transitional cell carcinoma. Drs. Paul A. Gikas of Ann Arbor and L. J. Cowdry of Milwaukee preferred the designation of urothelial c·areinoma. Dr. L. M. Franks of London f'ig. 5-R(: nal tissue with seatter~d tumor (ells (x120). considered it a case of carcinomatosis of the urinary epithelium. Dr. David Dahlin of discolored, and there were firm nodules beneath Rochester, Minnesota of:(ered. grade 4 squamous the capsule. cell carcinoma; Dr. W. Bullock of Los Angeles ~!so offered squamous cell carcinoma. Dt·. A. L. Vargas . ~.., Dr. Mostofi: The section shows kidney tissue of El Paso t.hought it is likely a tubular carcinoma. (Fig.5). Many islands of tumor cells are seen scat· Drs. C. Perez·Mesa of Columbia, Missouri and E. tered throughout; these are both in vascular Philippe of Strasbourg preferred metastatic car· spaces and in the tubules (Fig.6). The tumor cells cinoma. resemble transitional cell carcinoma of renal pelvis; however, no renal pelvis is seen. The sup· porting stroma shows edema, hyalinization and Sub~C(I UCnt History: Following surgery the pa· focal infiltration with plasma cells, lymphocytes, tient received radiotherapy. From June 30th to macrophages and occasionally polymor· August 4th, 1976, she r~ceived 4.500 R in the mid· pbonuclear leukocytes. plane of the left hypochondrium. In January 1977, the patient complained of abdominal pain, and 'fhe differential diagnosis here is between a there was some tenderness to palpation. Thore metastatic carcinoma and a carcinoma of renal was no weight loss and no radiogr.aphic abnor pelvis deeply infiltrating the renal parenchyma malities. and invading the blood vessels. Although no renal pelvis is seen, the transitional cell appearance of the cells is so pathognomonic as to make this Dr. Whitmore: The management· of this. lesion alternative the most likely diagnosis. Also the in· bears some similarity to the management of tran· tratubular spread is more in favor of this sitiona·t ceB tumors of the bla'dder. These tumors diagnosis than that of a metastatic carcinoma. can be graded and staged in a fashion quite Epithelial tumors of renal pelvis constitute analogous to their vesical counterparts; we would about 7 to 80,0 0f kidney tumors. They are consider this lesion a high grade, high stage tran· classified as are the epithelial tumors of bladder. sitional cell tumor of the renal pelvis, and the Their pattern of. growth may be papillary, prognosis in this setting is quite ominous. Our papillary with infiltration. or they may be sessile, survival rate after nephro·urete~ ectomy in pa· nodular, infiltrating, or non·papillary or non· tients with these lesions is something like 20% at infiltrating. five years. Or. Mostofi's Diagnosis: TRANSITIONAl, CELL CARCINOMA Histopathologic diagnoses submitted: Tr~nsitional ceU tardnorna ...... , 54 Renal cell ca.rdnoma ...... 11 Tubular adenocarcinoma . , ...... , . ... . 07 Squamous eeU ca.rcinoma ...... 03 Oth•rs. , ...... 09 Dr. 111ostofi: Although a few of the cells did have clear cytoplasm, the pa~tern of growth and the vascularity noted by Dr. Elkin do not exist in the slide that we saw. There are some areas of or interest is the question of whether or not Most of these patients die of local recurrences, pre-operative radiation therapy might be utilized most of which become evident within a year of the in situations like this. I tllink it has been clearly time of nephrectomy. I strongly suspect that this demonstrated that for high grade. high stage patient's loin pain may be a manifestation of local bladder cancer. pre-operative irradiation recurrence. The majority of patients die of local enhances survival after cystectomy in selected disease. although there may be metastases t.o the patients. This is not being systematically ex· bone, lung and liver. plored in this country. but I think there would be a strong argument for pre·operative radiation if you could accurately make the diagnosis in n Dr. Jorge Ceballos·Labut, Mexico City, Mexico: situation of this sort. Why did they do a myelogram on this patient? The first film shows contrast in the spinal canal. The problem with tumors in the kidney or in the ureter is that there is no way of pre· Dr. Elkin: I noticed that, but it did not seem operatively making an accurate diagnosis. One of pertinent, so I just kept out of it. Probably some the suggestions made in this regard is to use itchy orthopedic surgeon wanted it. urinary cytology us a means of grading these Or. John Campbell, Toronto. Ontario. Canada: tumors; the Scandinavians seem to be further Is there anything in these radiographs that make along with this than we are in this country. you exclude tbe possibility of squamous cell car· Another possibility is t.o get a tissue biopsy by cinoma? passing up brushes or stone baskets. getting ae· tual portions of tissue that can be examined Dr. Elkin: Nothing except statistics, I guess. histologically in conventional fashions. Squamous cell carcinoma usually begins in the pelvis on the basis of infection or calculi; there is As far as post-operative irradiation is con· no evidence of calculi here. There is no long cerned, the problem is twofold. First. there is a history of anything like leukoplakia, and there is possibility that your s urgical manipulations have luck of history of passing tissue shreds. disseminated the tumor locally outside the radia· Dr. J. Maxey Dell, Gainesville, FL: What tion field. Second, surgical manipulation could be would be the objection to a needle biopsy if it is expected to enhance the possibility of distant important to get the information for pre· dissemination from that site. You have really operative irradiation'/ opened up a field for potential dissemination from the diaphragm to the bladder, and you would Dr. Whitmore: Certainly. that Is one possibili· have to irradiate a much larger portal. ty; the probability of getting a good tissue diagnosis in a situation like this with our present There is rather unequivocal evidence, both techniques is really pretty good. The risk of the from experimental work and from clinical studies. needle biopsy is that of implantation; it has never that radiotherapy is more effective when given been precisely quantitated if that risk is worth pre-operatively. I n the high grade. high stage the benefits achieved. Only a clinical study would transitional cell tumors. whether of the ureter or provide an answer. U you do your aspiration with of the renal pelvis, the problem of multicentricity a proposed irradiation field. you could well argue seems to be admitted evidence; it is somewhat that you might offset the risk or implantation. less of a factor in the case of the low grade, low The Swedes have anticipated our using needle stage tumors. When there is a solitary papillary biopsy in pre-operative identification or renal cell tumor in the renal pelvis. the probability of its be· carcinoma; they have demonstrated no apparent ing associated with other tumors in the ureter Is adverse impact of needle biopsy on the prognosis appreciatively greater than when it is a solitary in patients with renal cell cancer. However, the high grade, high stage lesion. The patient who is number of cases is small, and the follow·up is most apt to have multicentric cancer in the blad· short. Most of our treatment failures in renal cell der is indeed the one who has low grade. papillary cancer are consequences of distant metastases; lesions; in tho kidney and ureter the same per·· that is why you do not see nny statistically tains. Much is being written now about the demonstrated improvement in survival from sim· usefulness of conservative therapy, that is. when pie nephrectomy over radical nephrectomy or can a resection of the ureter be done for lesions of [rom pre-operative irradiation over nephrectomy this general nature. You not only have t.o consider alone. The logical explanation is that the horse is the question of what the chances are of con· already out of the barn by the time a radical trolling that particular tumor, but also the risk of nephrectomy is done, and therefore, whether you getting new lesions in the urinary tract. The type do a simple or a radical nephrectomy makes very of lesion seen here is apt to be solitary. whereas little diiierence. In contrast with transitional ceU the low grade papillary lesions that may be seen tumors, local recurrences arc the principal cause in the ureter or renal pelvis are more apt to be of failure of treatment, failure to get the tumor associated with a tendency toward multieen· out without spilling it or getting its peripheral ex· tricity. tensions out. Thls provides a strong argument for 8 beneficial effects of pre-operative radiation, but it in incidence of local recurrences when radiation also increases the risk of possible adverse effect.s therapy is used. of needle biopsy. The irradiation that we have used bas been done through large pelvic portals (20x20 em) that Dr. Nei l Deeter, Lake Worth, FL: I would like go from the upper border of LS to the ischial to ask Or. Whitmore if. given the intraoperative tuberosities and out to the middle of the findings, he would proceed with a ureterectomy? acetabular fossa bilaterally, covering the whole pelvis. We deliver 2000 rads in five days, treating Dr. Whitmore: Yes. When a kidney is to be both portals daily, and then doing immediate taken out, the additional risk of ureterectomy is cystectomy. The other program in which we bad so trivial t,hat we ordinarily would do it. On the experience was 4000 rads given in four weeks other hand, in an elderly patient who represents a through somewhat smaller portals; cystectomy somewhat ha~ardo us risk, we might elect to take was delayed four to twelve weeks after the com as much of the ureter as we could reach and leave pletion of radiation therapy. Both of these pro· the rest of it, not making a separate incision to grams have essentially produced identical results get the rest of it. in terms of. improvement and survival in patients with deeply infiltrating bladder cancer; both have Or. Wallace Buchanan, Fort Lauderdale, FL: produced the same reduction in the incidence of Would Or. Whitmore comment on time-dose rela local ~!'lcurrenees . Others are using a variety of tionships in radiotherapy of this tumor? · programs. lt is important that we do not draw . ~ premature conclusions about what is optimum in • • I Dr. Whitmore: I have to use the bladder ex· that regard. perience as a basis because we have not utilized Dr. Brigit van der Werf of Rotterdam has used pre·operative irradiation in transitional cell a technique of suprapubic implantation of radium tumors of the upper urinary tract. There are needles in selected patients with bladder cancer. three basic reasons why pre-operative radiation She has found an incidence of about 15% therapy might be beneficial. First, ~here is cer suprapubic wound recurrences wh en no radiation tainly good evidence now that relatively modest therapy was utilized. I think she used 1500 rads doses of radiation therapy (4500 to 5000 rads of given in four or five days as post·operative radia four and a half to five weeks) have the potential of tion; the recurrence rate was reduced to about 2 destroying micrometastascs in regional nodes: or 30,\), When she used 1050 rads given in three that is true for testes, breast, bead and neck le· fractionations immediately prior to the implanta sions and cervix, and it is not Wogical to think tion. suprapubic recurrence rate was reduced to that the same might pertain to bladder and 0%. As soon as we complete the obligations of our similar tumors. present protocol, we may well go to smaller doses Second, experimental evidence elearly sup· with more intensive irradiation, something on the ports the concept that it is the peripheral, well order of 1050 rads in three days, and then im· oxygenated extensions of the tumor that are most mediate operation. We are not trying to cure the susceptible to radiation therapy, so if you have tumor with radiations, just trying to reduce the local extensions not completely within the field of prospects of local recurrence from peripheral surgical excision, i~ is conceptually logical to local extensions and from the distant dissemina believe that ~hese might be favorably innuenced tion as a consequence of surgical manipulation. by pre-operative irradiation. We are no ~ trying to sterilize the regional nodes because we are doing a routine node dissection. If A third factor is that of the potential ill crfccts you are .trying to sterilize regional nodes with or s ur ~i ca l manipulation, whether it be local radiation therapy, you have to go to higher dissemmation in the wound, or actual lymphatic dosages. vascular dissemination as a consequence of s urgical manipulation. If you can pre-operatively give the ~umor a sufficient dose to reduce the Dr. Leonard Shuko,•sk)', Tampa, FL: The viability or the number of such cells, then you will studies lrom Anderson show the five-year sur· statistically reduce the probability of distant or vi val to be 500.1> ; the study from Memorial yielded local dissemination. No one, to my knowledge, has a 5-year, 40% survival rate. The difference bet yet established, even theoretically, ~he optimal ween the two studies is the higher dose used at time-dose relationships. From published as well Anderson where no lymph nodes are removed at as our own experiences in patients with the worst the lime of surgery. The survival is just as good. bladder cancer (B2 and C lesions, T3lesions of the UICC system), survivals are approximately dou· ble following a combination of pre-operative ir· Dr. Roger- Byhardt, Milwaukee, WI: I wonder radiation an d cystectomy, than what they were if Or. Whitmore would comment on the complica without the use of pre-operative irradiation. T his tion rate of the series with 2000 rads in a week as is a true effect: there is roughly a 50 °.1> reduction compared to those who had 4000 rads. 9 Dr. Whitmore: The one complication that we persistent or recurrent disease within a relative clearly recognize as sequel to radiation therapy. ly short period ofthree to four months. Their data whether 4000 rads or 2000 rads, is the delay in would suggest that cystectomy in patients who wound healing if infection develops. With that have persistent or recurrent tumor at tho end of single exception, I would have to say that radia· three to fou r months may be the optimal therapy. tion therapy in general is well tolerated as it is T here are some increased complications in pa· given and not associated wi th any clearly tients who have received 6000 rads. some rise in definable increase in mor tality or morbidity. Pa· operative mortality. but by no means prohibitive. tients will get diarrhea while they are convalesc U that were clearly demonstrated to be the ing from surgery. The tubes are still in, and they superior program, I certainly think there would are still on intravenous administrations; thus. be no objection on the basis of the complication or they complain bitterly about it. It does not create mortality rate. any metabolic problems and usually subsides within three to seven days. It is a very obvious Dr. John Har per, Fort Lauderdale, FL: It has event that can be attr ibuted to the radiation been our clinical im pression that the effects of therapy, but it does not prolong hospital stay. It radiation therapy go beyond just three to four is a nusiance to the patient but no great problem months. I wonder if there might be some question to the physician. as to whether or not we should go ahead with cystectomy in the presence of demonstrated Dr. del Regato: Discussions of radiotherapy by tumor after such a relatively short period as those not specifically qualified tend to be· three to four months. simplified to the statement of a dose, 2000 or .. 4000, as being the meaningful factor; in reality, Dr. Whitmore: Agreed. It has to be somewhat the time in which these doses are administered is arbitrary. Ther-e is no question but that some pa all important to its effectiveness. Very often a tients will still be undergoing regression of their smaller dose in a longer period of time is more ef· tumor after a t hree to lour month period; per· fective than a larger dose in a shorter time; sistenee of tumor after three to four months does preference for a longer lime of irradiation may not necessarily mean it would be there a year imply a larger dose but a lesser intensity of treat later. I think 1 am quoting Luther Brady correctly ment. when I say that he has seen patients who have gone as long as two years before all evidence of Dr. Paul Berger, Dunedin. FL: Does Dr. Whit· tumor has disappeared. However, I would be a lit· more have experience with a protocol of 6000 rads tie reluctant to sit £or two years waiting for it to in six weeks, withholding cystectomy until go away. I agree that the three to four month evidence of persistence or recurrence? period is an arbitrary one. and that is one of the problems that needs to be resolved. You would Dr. Whitmore: No. The only experience we like to be able to determine which patients arc go have had with 6000 rads has been in patients who ing to respond. A justification for restudy of 6000 come to us as radiation failures; whether they had Tllds would have to be based upon evaluation of recurrent tumor. persistent tumor, or new tumor. s uch things as urinary cytologies during the there is no way of knowing, nor do we know from course of the pr ocedure. looking lor something what total population of patients these were that would he I p pick out retrospectively that derived. Our selected experience in that category population of patients who would ·yield a good of patients merely tells us that salvage cystec response to radiation therapy. We throw them all tomy is clearly worthwhile. Bloom and Wallace in into the same pol and treat them; some get bet· London are doing a study in which patients who ter. and some do not. Until we learn to look at have had full CO\jrSe radiation therapy are closely something intelligently and in a prospective way, observed and subjected to cystectomy if there is we are wasting·our time. 3. TRANSITIONAL CELL CARCINOMA OF THE KIDNEY PELVI S Cont.ributed by J. F. Wilson, M.D., A. J. MacGillis, M.D. and M. W. Elson, ~I.D., Milwaukee, Wisconsin The patient was a 47-year-.old woman in Mar ch was then associated with pains in the left flan k. 1976 when she complained of lumbosacral pain; a On examination she appeared pale and had a diagnosis of lumbosacral herniated disk was palpable large left-sided abdominal mass. There made, and she bad a laminectomy. Three months were 20.000 white cells per mm•and marked shift later, she complained again of severe pain. which to the left. 10 Dr. Elkin: Most striking in the scout film is Lhe very large soft tissue mass in the left flan k pushing on the greater curvature of the stomach and extending to the level of the crest of the il· ium. I see no calcification in the mass (Fig. 1). On the right sid e or the urogram, t here is good excr etion or contrast modium wi t h t he ap· pcarunce of spread of t he middle and lower calyceal groups; t his could be due to a prominent renal column or to a mASS such as intrarenal cyst. However, this does not seem t.o be pertinent to the present discussion. On this film there is no opacification of c:alyees. I see curved lines or opacity in the upper portion or the mass on the left side; I consider these lo be curvilinear collec· lions of contrast medium rut her than artifacts. These would be r ims or tissue about large cysts or rims in a marked hydronephrosis. This is a very lar ge mllS$, and it is diffjcult for me to think that iL was overlooked thr ee months p1·eviously when the paLien~ was in a bospital for myelography and laminectomy; I will assume that this mass appeared within the past three months, although I can think of no renal neoplasm that would grow this large so quickly. Could this be a result of her operation? Injuries to the urinary tract have occurred during laminectomy. During Fi,g. :!-Cro;;rrnm htilin~ tu )>htt"" hmctiun or lh<· lert kidne)'. curetting of the d<•gcno•·ated disc, the rongeur Ur inoma wc)uld thus be my first diagnosis, has been known to slip throug h the anterior limit alt hough the extent or hydroncph•·osis (as judgod of t he int er vertebral spuce wi th lacerat ion ol' the by t he l'ims of contrnst medium) does seem large ureter, which lies in close approximation to tho for obstruction of less tlmn t hree months (Fig. 2). L4 and L5 vertebr al bodies. If this happened, sho might have a large urinoma causing obstruction Suppose t h:lt my nssumption was wrong, t hn~ to the kidney with marked hydr onephrosis, and the mass was present three months ago bu~ was this would explain the rims of contrast medium missed, or the patient was nol told about it. I you see on the urogram. The urinoma could be in· would then think of some peculiar type of cystic fected, and this would explain the leukocytosis. lesion that has probably been there a long time. Multicystie kidney can be first discovered in adult life, and it h:1s been reported that occn· sionally co ntrast medium rims can be seen at urography, expecially if a hig h dose of th e con· trasl medium was administer ed. Another possi· bility might be a lnqrc multilocular cyst, con· sidered by some writers as a cystic hamartoma and by others as a benign type of Wilms' ~umor. Similar to multilocular cyst is the rare entity caUed "polycystic nephroblastoma;" this may be Lhe same lesion reported by others as multi· locular cyst. Polycystic ncphroblastoma is sup· posedly a well·diffcrcntiatcd nephroblastomn with multiple epithelial cysts, either of low·gr:1de malig nancy or possibly even benign. Dr, Elkin's Impression: 1) URINOMA plus IIYDRONBPIJROSlS 2) ,\llJt:rn.OCULAI! CYST ~) POLYCYSTIC :-.'EU ROBLASTO)IA Radiologic impressions submitted: Hyd ronephrnsi$ .... - 31 Pyeloncphrili.s , ..- .. 15 Perinephric :tb.!;ee~!;...... 14 Cystic kidn~y ...... 08 Renal vein thrombosi~ ...... 07 Transition:d t ell carcinoma...... 06 Renal cell c~trdnom:i...... 05 Others ~ ~ ...... 12 Ur. ·Elkin: I am sure hydronephrosis was sug gested on the basis of tbe crescents seen on the · urogram. There is nothing that would make me think of pyelonephritis radiologically. Perinephric abscess would not explain the crescents. Cystic kidney ·without defining the type of cyst is fine; these rims of the tissue can be s.een t·adiologically in various types of cysts. You do get a big kidney in renal vein thrombosis, although t his would be pretty big for thal. You may see a ·prolonged and delayed nephrogram in F' ig. 4-Hcplacl'mcnt c.•f ~;u rfacc epithelium by ntllplastie cells renal vein- thrombosis, but I see no reason for (x1(i0). thes-e crescents. Transitional cell carcinoma is a good diagnosis; if this patient does have seen in the center of which there are a few hydronephrosis ;•nd we are thinking of a reason' atrophic rerial tubules and fibrous tissue and in r. for it, then a transitional cell in the pelvis of the orie area mature fat cells and thick-walled vessels . ' junction area would be a good reason for it. If it is probably representing peripelvic fat. The tissue a renal call carcinoma, it has to be a par.enchymal is obviously from the wall of a cavity {Fig.3), and lesion that has involved the renal pelvis, causing since both sides show epithelial cells, it could obstruction and resulting in hydronephrosis. represent a papilla. The surface epithelium is There is evidence of either hydronephrosis or replaced by a layer of neoplastic cells arranged in cyst of the kidney, and if you want to pick a cause, micro-papillary and tubular patterns perpen the transitional cell might be the best cause, but dicular to the basement inembrane {Fig.4l. A thin there are other things that might cause it too. I layer of Oattened epithelium partly covers the have not given up on the urinoma. surface. In other areas the cells are piled up (Fig.5l. Or. del Regato: Drs. M. W. Elson and A. C. The differential diagnosis is between a cystic Sp~>ranza of Milwaukee offered an impression of renal cell carcinoma, a renal cell carcinoma aris renal cell carcinoma. Dr. T. Ishikawa of Cincinnati ing in a cyst. and a transition.al cell carcinoma. In suggested ureteral obstruction and hy cystic renal cell carcinoma, we have cystic areas drOliephrosis. Dr. J ..F . Hernandez·of Mexico City within an otherwise typical renal cell carcinoma. offered renal abscess or pyonephrosis. In renal cell carcinoma arising in a cyst, there is a distinct cyst wall and either solid sheets of clear Operative Findings: On July 16, 1976, a large or-granular cells, or cells can form one of the hydronephrotic left kidney was found; 1400 cc of various patterns seen in renal cell carcinoma. clotted blood were evacuated and a nephrectomy None of these features· is seen in this case. In· carried out. The kidney was markedly enlarged, stead, we see a layer of flattened epithelial cells meas ured 14 x 24 em. A final diagnosis of overlying either piled up epithelial cells or tumor hemohydronephrosis l"as rendered. cells arranged h1 a micro-papillary configuration. The radiological diagnoses of hydro- and Dr. Mosliii: The. orientation of these. sections pyelonephritis would certainly support thi.s makes interpretation difficult. A piece of tissue is diagnosis. Fig. :> - Thin layer or (lallened !)ur(a:ce e:pitltclium (:d90). Dr. Mostoll's Diagnosis: February 8, 1977, she was hospitalized with TRANSITlONAL CELL CARCINOMA IN pulmonary metastases. HYDRONEPHHOTIC KIDNEY Dr. Whitmore: It would be nice to know Histopathologic diagnoses submitted: whether the hydronephrosis was the cause of the Renal cell C.lrc:inoma ...... ~ .. . 36 tumor or whether the hydronephrosis was an in· Tran.silional cl!ll c:atc.lnoma ...... l6 Angiosarcoma. . , ...... 08 cidental finding and the tumor was in the renal Organi~ed htunatom.a . , ...... 07 pelvis. As I listened to the presentation, the ques· Olh•rs ...... 20 tion I had was whether the hydronephrosis was caused by the tumor or whether the patient just happened to have an incidental transitional cell Dr. Mostofi: I have already discussed the tumor in a kidney that was hydronephrotic. I reasons for this not being a renal cell car cinoma. I gather there was no large bulky tumor in the do not really see the diagnosis of angiosarcoma renal pelvis. The vast majority of renal pelvis unless one were to interpret those cells forming tumors do have hematuria as a presenting symp columns and cords as forming vascular spaces, tom; in the patient who had a marked but they are not doing that. There is hemor· hydronephrosis from such a lesion, one might an· r hage in the cyst. but this is not the picture we ticipate that there would have been a long· see in organized hematoma. standing history of hematuria with progressive dilatation. In the situation where t here is no clear Dr. del Regato: Or. Fantelli of Clevc(and and pre-operative assessment of the grade and stage Dr. F. M. Enzingcr of Washington, D.C. also made of the tumor being dealt with, nephroureter· a diagnosis of transitional cell carcinoma. Dr. C. ectomy is a proper approach. Rich of Cleveland and Dr. Maria Viamontc of Miami preferred renal adenocarcinoma. Dr. Leo References: Lowbeer of Tulsa preferred angiosarcoma. Dr. J. Boggs, t..K., and Kimmelstiel. P,: Benign mult.iloculur (yitic B. Frerichs of El Paso also thought of a vascular nephroma: Rer:ort of two c.Mes of so·caUed multilocular cy!t of tumor but not Kaposi's. Dr. P. Walter of the kidney. J. Urol. 76:530-541 , 1956. Strasbourg preferred hemangioma. Cbrjst., M.L.: Polycystic ncphrt">blastoma. J. Urol. mS:570·575, 1967. Oatnow, B.. and Da ni el. W.W., .Jr.: Polyc)"!tic: Subsequent History: Following operation, the nophroblostom•. J. A.M.A. 236:2528·2529, A976. patient did poorly, lost 18 lbs. in weight and Kern, li.B .. Barnes. ·w. . and Malament, M.: Cumbar laminec· developed a supraclavicular adenopathy. On t.omy and associated u rct~ral Injury. J. Urol. 102:675·077. 1969. 4. WILMS' TUMOR IN AN ADULT Contributed by Harold G. Jacobson, M. .D. , Bronx, New Yor k The patient was a 24-year-old man in May 1975 per part of the renal mass. The mass is fairly when he gave a history of left flank pain of three large, measuring about 9 em x 8 em in width. It is weeks duration, accompanied by fever and pushing the upper part of the ureter medially. In nausea. On examination a 5 em tender mass could general the mass is hypo vascular. be palpated in the left lower abdominal quadrant. The available film of selective injection into the lower left renal artery is unfortunately a left Dr. Elkin: T he history in this case spea.ks for posterior oblique pr ojection, and thus we are an inflammatory process. The patient is young, looking at the mass from a di!ferent angle and the duration of symptoms is short, and he has eannot correlate our findings easily with those of fever, nausea and left flank pain. A tender mass the other angiogram. The lower pole vessels are was palpated on the left side, so ~he diagnosis deviated by the mass, and there are clusters of should be renal abscess. All we have to go on are abnormal ve.ssels at the lateral aspect of the two films of selective angiography, and we get mass. There are capsular vessels extending below two films, only because the lef~ kidney has two the expected level of the lower border of the main renal ar teries. kidney. There is no AV shunting or puddling of On selective injection of the left renal artery, contrast material (Fig. 21. the upper pole of the kidney looks normal. This could be a renal abscess, but I think not However. the intrarenal vessels of the middle and because the peculiar vessels seen in the mass lower parts of the kidney are deviated by the would be unusual. This is a hypovascular mass in the lower pol ~ of tho kidney (Fig. 1). neoplasm, despite the young age of the piltient. There are several faint abnormal vessels in the Watson et al reviewed 100 consecutive casos of mid portion of the kid ney, apparently in the up· hypernephroma. finding that 6'\b were avascular 13 be unusual for a renal abscess. A papillary tubular adenocarcinoma is a feasible diagnosis; I cannot object to that possibility because I think the vessels we lend to see in the renal tubular adenocarcinoma are not these stretched elongated ones we see in sarcomas or Wilms'. I would exclude simple renal cyst; there arc vessels going through this lesion. both in the AP and obli· que views. that do not go through cysts. A Jym· phoma is a possible diagnosis radiologicl•lly. I do not know whether or not Dr. Mostofi reels a lym· phoma is a primary t umor of tho kidney, but radiologically, a lymphoma can appear as a single mass simulating a primary tumor, most common ly as multinodular lesions, or we cnn see it in vading the kidney from the retroperitoneal lymph nodes. Dr. del Regato: Or. Manuel Viamontc of .Miami also suggested a lymphoma. Dr. H. Ramirez· Jimenez of Mexico City offered an impression of angiomyolipoma. Operati"e findings: On May 30, 1975, a left nephrectomy was carried out through a posterior incision; perinephric fat and para-aortic nodes were also removed. The specimen measured 15 x 10 x 7 em and presented n hcmorrhagit 11nd Fig. 2-Lt•rl POSll'l'ior oblique J)rojt•(•tion of t~e l c•Cti\'C itngiogtn.ph)' ~) r tht• lciWl'r n•nal Hrll:r~·: ralnt \'(•S;o,l• l!l In h) w~ r rJnl u mw;..o;. Fi~. 1- Selctchc ;.lngingraph.y of thf' upper pol~ renal Arh•t)'i mtt"" In lu~('r SlfJic. and 16% were hypovascular." Also. about 5% 01 renal adenocarcinomas are of the papillary· tubular type. and they are hypovascular. If l hnve to state an order of Jlrcfcrcnce. I will make sarcoma my first diagnosis and ndult Wilm s' tumor rny second diagnosis, but I cannot I'll Ia out the other neoplasms T mentioned. Dr. Elkins lmpr(•ssion: l) WILMS' TUMOR 2) m :SENCIIYMAL 'l'VMOR ~) I.Yi\IPIIOMA 1[) IIYPOVASCULAR ADENOCARCINOMA Radiologic impr~ssio ns submitted: Rtnal earbunc!e or abscess_...... -· .. 3'9 Renal eeiJ ~arejnorna ... _...... 15 Simplt- ren31 t~'St...... Oij t.ymphoma • ...... 06 Va.riou~. malignant ...... l2 Variou.t, benign ...... IS Dr. Elkin: I think that carbuncle or abscess is n legitimate thought. especially when the history is as it is. However. I would exclude that; I think lhal the vessels going through the lesion would renal pelvis, and the cells do not resemble transi tional, squamous or undifferentiated epithelial cells. We are then left· with the diagnosis of either atypical Wilms' or some sort of sarcoma. No spindle cells are seen, and the tumor con· sists entirely of undifferentiated cells that are most reminiscent of primitive clements of Wilms' tumor (nephroblastic cells); the majority of par ticipants would certainly diagnose this tumor as atypical Wilms'. It may be argued that it should be designated as undifferentiated malignant tumor. It should be mentioned that such tumors may show typical Wilms' tumor either in other areas or in the metastases. Dr. Mostofi' s Diagnosis: ATYPICAL WILMS' TUMOR Histopatilologic .diagnoses submitted: Wilms' tumor ...... ~ ...... 35 of ·.J Transition!\) tell .;.ilr<:inoma...... , 15 '· ,-I Sarooma ...... ~ ...... 14 Hcmangiopcricytoma ...... 08 Othors...... 16 Ur. Mostofi: My own diagnosis was atypical Wilms' tumoe with predominance of mesen chymal elements. I am happy to see that the ma jority agreed with that diagnosis. We can dismiss Fig. 4- UndiHtrtntiated tumor cells with Jiltle cytoplas m ~x:!-.10). necrotic area. about 4-cm in diameter in the lower pole of the kidney; the renal vein was filled with necrotic material. Dr. Mostori: The slide shows a tumor occupy ing the medullary part of the kidney and extend ing to the peri pelvic tissue. No definite capsule is seen; the tumor is infiltrating the substance of the kidney (Fig.31. The tumor cells are undifferen tiated, intermediate in size, with round or oval nuclei that have a delicate chromatin pattern and rare nucleoli. There is little or no cytoplasm (Fig. 4). The cells occur in solid sheets, but occasionally there is a suggestion of tubular structures. The stroma is scant but well vascularized. Several areas of hemorrhage are seen. The possible differential diagnoses here are a renal cell carcinoma, a transitional cell carcinoma of renal pelvis, an atypical Wilms', and a sarcoma of some type. No clear or granular cells and no tubular struc tures_of any type are seen, so rena.! cell carcinoma can be excluded. AlthOugh the tumor is in med ullary portion, there is no radiological or pathological evidence of origin or involvement of transitional cell carcinoma without too much trou Or. Whitmore: Well, the unusual feature of ble. Sarcoma is justified except for the presence .this case is the presentation with fever; whether of tubules that I have called attention to. or not that is a "red herring" or whether that is Hemangioper icytoma is not justified in this case an important part of the disease or not is because we do not see the vascularity. debatable. In children with large Wilms' tumor, fever is a well recognized feature. This is a Dr. del Regato: Drs. P. A. Gikas of Ann Arbor r elatively smaU lesion. The age of t he patient is and J. Watts of Cleveland also made a diagnosis certainly unusual for this diagnosis, but over 100 of Wilms'; Dr. F. M. En zinger of Washington, D.C. cases have been reported in ndults. The diagnosis called it atypical. Drs. H. A. Azar of Tampa and A. of Wilms' tumor in adults should dictate the same 0. Severance of San Antonio preferred poorly dif type of multimodal therapy as in children, but it is ferentiated transitional cell carcinoma. Drs. D. not very well defined due to limited experience. Dahlin of Rochester, Minnesota and R. L. Font of The hazards of such therapy are relatively Washington, D.C. called it an undifferentiated minimal; the prospect is that they would respond sarcoma. Dr. Y. LeGal of Strnsbourg offered rhab $imilarly. As far as therapy of metastatic disease domyosarcoma; Dr. M. Weiii-Bousson, also of is concerned, the possibility of surgical excision of Strasbourg, preferred fibrosarcoma. an isolated or l imited metastatic disease of the lung is the most favorable; however, even liver metastases have been successfully resected in pa Subsequent llist.ory: Following operation, the' tients with Wilms' tumors. patient received radiotherapy and Vincristine. In January 1977, he had developed lung metastases References: WAtson, R.C .• FJeming, R.J .• •nd £vans. J.A.: Arterjogtaph- v in and was receiving chemotherapy. thto: diagnosis of renal <:arc:inoma. Radio4ogy 91::888$7, 1968. 5. WILMS' TUMOR IN AN ADULT Contributed by ft. Isbell, M.D. and ~lanu c l A. Carta, M.D., Tampa, Florida The patient was a 29-year-old man in March lower pole. Blood supply to the tumor is from in 1976 when he developed fever and pain in the trarenal arteries as well as from the branches of right flank. On examination there was a palpable the capsular artery (Fig. 2). mass 7 x 9 em in the right side of the abdomen. The angiogram suggests solid neoplasm with Or. Elkin: There is a fairly sharply bordered tumor vessels but not the wild vessels of mass- occupying most of the lower two-thirds of hypernephroma. The smooth outline of t he tumor the right kidney and extending about 2 em and lack of encasement of vessels speak against beyond the lateral border of the kidney; the mass transitional cell carcinoma involving the paren is relatively radiolucent as compared to the densi chyma. This could be a big adenoma or could be a ty of the excretory nephrogram. The mid and lymphoma. The distinct and attenuated vessels lower calcyes opacify only poorly, and the upper seen cspeciall'y in the capsular region are the type calcyes are pushed by the mass. The kidney ap of vessels seen with sarcoma or Wilms' tumor. pears plump, but its borders are well defined, The tumor vessels seen in Wilms' tumor have despite the mass extending beyond the kidney .Fie. 1-lnlrtxenous pytlogram ~howlng ma."""' OC'tupyi_og lo•·u (Fig. 1). IW'I'Ihird.s of right rt l dn~r. In the selective right renal angiogram. the vessels are stretched by the mass, but there are also abnormal vessels in the tumor with some puddling of contrast medium. The striking thing is the extension of the mass around the kidney along its lateral lower border with displacement of perforating capsular vessels by the extension of the mass with a number of abnormal branches. In addition there is a smoothly outlined tongue of this extrarenal extension of the tumor capping the lower pole of the kianey. The cortical nephrogram is disturbed along the lateral border of the kidney but surprisingly not in the lower pole, despite the extension of tumor around the tion of cont•·ast material from the adjacent calix. Radiolog i c<~ lly, <~ngiomyolipoma is usually a vascular -lesion: it is more common in elderly women. However, ~he major thing that did not make me consider an angiomyolipoma was the lack of vt~scularity. Most of the cases we have seen mi mic in .;orne-r e~pcds the florid vascularity of a l'enal cell carcinoma. Dr, del Regato: Drs. A. C. Sper an~:. of Milwaukee and M. Viamonte of Miam i also sug gested Wilms' tumor. Dr. B. Weiss of Du nedin, Florida offered renal cell ca .rcinomt~ . Opcmtivc Findings: On March 31.• 1976, a right nephreCtomy was carr ied out. The specimen measured 12 x 10 x 7 em and it contained a tumor 8 em in diameter, which bulged on the perinephric fat. D•·-Mostofi: Part of Lhe kidney is destroyed by a very cellular tumor \V hich is lobulated. In some areas the cells are large. [airly uniform with predominantly oval vesicular nuclei itnd a small nucleolus. T he cytoplasm is scanty. Scattered throughout are the immature wbules lined by high cuboidal cells (Fig. 3). The third cell type con sists of small cells, also with little or no cytoplasm Pig. 2-S<' It cfi\•t~ right rcrt:ll angi(•gram showing iHrt'1Ched a0d nuclei of various forms (Fig. 4). In some areas ''(;!)$(·1~. these cells are arr anged loosely; in others they been descr ibed as "distinct," "attecnuated," "zig f'i~. :l-K i drh.'~' par ti;:il l ~· dt•$lron.d by IUntur: fai rl ~· uniform zag," and among t he epicureans as "spaghetti l'l~ ll s wil h scan•ly (_'yluJ•1a~m 6:2·10). · like." The appearance of ~he tumor vessels in this patient fits all of t hose terms, and hence my first choice ·is Wilms' tumor. However, my first reac tion wo~ld b.\' "~he p~~ien~ need$ surgery.'' Dr. Elkin's Impression: I) W-ILMS' TUMOR 2) SARCOMA ;l) LYMPHOMA Radiologic im11ressions submitted: Renal celt carcinom:l ...... 38 Dr. l~ lk in : That vascularity is not l.he Lype t hat I would expect in the ordinary renal cell car cinoma, even in the bypovascular tubular renal cell carcinoma. The reason fo r t he diagnosis of perinephric abscess is t hose peculiar· vessels spread beyond the border o[ t he kidney.Aiso, the outline, t he border of the kidney, was indistinct: t hose are two findings you do see with a perinephric-abscess. However. t he mass we saw on ~he kidney wt~s quite smooth: it extended almost ~o the region of the superior calix. A big ren~ l abscess with perinephric extension would have. produced some interference with l,he excre- cell population and the presence of distinct tubules· make the diagnosis of sarcoma untenable. The histology·ful£ills the criteria for a diagnosis of Wilms' tumor. Tliere are undifferentiated nephroblastic elements. spindle-shaped cells and albeit· primitive distinct tubules. This diagnosis is made even though no preglomeruli or pseudoglomeruli are seen. In this in~tance, the tumor is predomin·antly mesen.chymal. This category of Wilms' tumor has a less favorable prognosis than the predominantly epithelial type of nephroblastoma. Dr. Mostofi's Diagnosis: WILMS' 'J'UMOR, PREDmUNAN'J'LY MESENCHYMAL Histopathologic diagnoses submitted: Wilms' tumor ...... , ...... 57 Various sa.roomas...... , . 12 Carcinoma ...... 09 Others ...... , ...... , .. ll .. Dr. .1\fostofi: The diagnosis of sarcoma is jus~ified until you see the tubular structures or proglomerular s.tructures. I think we have said enough about carcinomas and others. Dr. del Regato: Sister Ignatius of Cincinnati and Dr. G. Farboody of Cleveland also made a diagnosis of Wilms' tumor. Dr. L. M. Franks of London considered this case not unli.ke the previous one except that he could see no differen tiation to urinary epithelium; he questioned the possibility of neuroblastoma. Dr. Komorowski of l~ig. 4-'fuooor c(m!'iHiing nf lno"cly arnmged tells (xlO()), Milwaukee offered leiomyosarcoma and Dr. S. E. form solid aggregates. At times the cells are. Vernon of Los Angeles, metastatic embryonal spindle-shaped and hav~; an eosiMphilie tumor. cytoplasm. There is considerable focal edema and Subsequent Ilistory: o·n December 7, 1976. the scattered lym~hocytic infiltration. patient was well with 'no evidence of re· The differential diagnosis possibilities are curre·nce or metastases. He is receiving neuroblastoma, various sarcomas and Wilms' chemotherapy. tumor, predominantly mesenchymal. Dr. WhU.more: This presentation with flank Since there are no Homer Wright's rosettes pain and fever is unusual in my experience; and there are (listinct tubular structures, the perhaps in young adults who present with flank diagnosis of neuroblastoma can be readily exclud pain and fever and have a renal mass, the ed. Although the cells are sometimes spindle diagnosis of Wilms' tumor ought to be more shaped. suggesting a sarcoma, the pleomorphic seriously considered. · 6. ANGIOMYOLIPOMA OF THE KIDNEY Contributed by H. Ramirez, M.D., H. Cruz-Ortiz, M.D. and J. Ceballos-Labat, M.D., Mexico City, Mexico The patient was a 44-year-old man in hemoglobin was 10.5 gm%, the urea nitrogen 15 September 1976 when he complained of rhgmOfo and total proteins 5.9 gmO,b, hematuria accompanied by lumbar pain and loss of weight. One year previously, he had suffered Dr. Elkin: I cannot see the right kidney on this trauma to the left flank, followed by hematuria of film. The selective left renal angiogram has a short duration. On examination there was a large good deal of aortic reflux for some reason with ·mass in the left side o{ the abdomen. The opacification of .superior mesenteric branches and 18 that the mass is a solid tumor containing adipose tissue; it has big feeding vessels. My first diagnosis would be retroperitoneal liposarcoma. If the big· feeding vessel we saw just above the renal artery is actually branching from the renal arterv (which I doubt), then we could talk about its being a big capsular vessel and suggest that the neoplasm had its origin in the renal capsule. Dr. ~Elkin's Impression: 1) ARTERIOVENOUS FISTULA 2) RETROPERITONEAl, LlPOSARCO~I A Radiologic impressions submitted: Arturiovenous fistula . , ...... 80 Ret:roperil,oneat hematoma...... 15 Renal ceU carcinoma...... l 2 Rclro peritonett1 turi\or. , . . . . . , . . QS Angiomyoli!Joma . .. .. , . . . , ...... 05 Renal vein thrombosis , , ...... , .05 Others · ~ · ...... , . •...... 12 Dr. Elkin: I think the patient has two arteriovenous' fistulas. However, I do not t hink the pathologist is going to be able to make that diagnosis from the one slide he received, so I do not care what he says. The patient had an arteriovenous fistula. The patient might have had a retroperitoneal h.ematorna in addition. but the retroperitoneal hematoma would not explain Fig. t- SeJl'cti\'4.' left renal nngiogram with an appartnt anerio· those .Prominent vessels feeding the mass. With v~nou!(l ristuJa. regard to renal cell carcinoma, it appeared to me that this tumor was being fed, not by branches of arteries of the celiac axis. I cannot say much more the intrarenal vessels. but rather .by lumbar or a'bout that. The left renal artery is big, and there capsular vessels; this would go against a is early filling of the renal vein, or what I take t.o diagnosis of renal cell carcinoma. It is a tumor of be renal veiq, and a lot of funny vessels (apparent the retroperitoneal space. Angiomyol.ipoma is an ly venous) medial to the kidney. Initially, I interesting suggestion. An angiomyolipoma does thought that the opacified tubular structure be contain fat, and we usually think of it as being tween the bifurcation of the main renal artery is something in the kidney, but I have seen one case renal pelvis, but I could not make out any calyces. in the hilum, extrarenal hilum, of the kidney. We or ureter, so I thirik it is renal vein. I will call this have aloo had one recent case that actually did an arterioven.ous fistula in the kidney and at· come Irom the capsule of the kidney. I did not tribute it to the left flank trauma of one year ago. think of that, but it is a possibility. A renal vein I wonder whether it was penetrating trauma. thrombosis would not explain the peculiar vessels I get the impression of lucency to the left of the we see nor what I think is lucency. lumbar spine; the left kidney appears pushed up Dr. del Rcga!o: Dr. Paul Berger of Dunedin, and the aorta deviated to the right. The S!Jperior Florida 'also suggested a retroperitoneal sarcoma mesenteric artery and its branches are also push· or an arteriovenous malformation. ed to the right. I think that there is a large ill· defined mass in the left flank. There are a number Operative Findings: On October 16, 1976 a le.ft of large and peculiar vessels just to the left of the nephrectomy was carried out for a large tumor. lumbar spine, extending to the level of the No enlarged nodes were found (Figs. 2 and 3). sacrum, apparently feeding the left-sided mass. These vessels come from a large artery just Dr. ~fostof i : The kidney is essentially normal: cephalad to the renal arterY.. This large artery is !or the most part, it consists of interlacing bands probably a lumbu vessel rather than a capsular of spindle. cells separating. many thick-walled tor artery (Fig. 1). tuous blood vessels (Fig. 4); these spindle cells are First.- there seems to be an arteriovenous plump and co.ntain cigar-shaped nuclei. Scattered fistula in the kidney; I will say that it is traumatic. throughout are islands of rna~ure fat cells (Fig. 5). There fs a retroperitoneal mass; it is very big and In areas the cells are pleomorphic with hyper relatively radiol ucent, at least in part. I think chromatic nuclei and occasional giant cells (Fig. 6). 19 The blood vessels vary considerably in thickness. to one. T he presenting symptoms at·e shock Some are very cellular·, others hyalinized and still (seco:ndary to massive retroperitoneal hcmor· others dilated. rhageJ. hematuria, acute abdomen, and/or mass. In the presence of exuberant leiomyomatous The ages range from 8 to over 80, hut most occur component, t.hc cellular pleomorphism and giant between 20 and 50. Patients may carry the cell s often mislead to a diagnosis of sarcoma, and tumors for many years un til r enal failur e or dtlpending on t he predominant component, to h(•morrhage call attention to their presence. either leiomyo· or lipo·sarcoma. The com bination of the three elements, muscle, fa t and tor· Angiomyolipomas are often present in patients t uhus blood vessels, is . pathognomonic of with t u.berous sclerosis complex; in these in angiomyoli poma. In over 300 angiomyolipomas in stances they are usually multiple and bilateral. the American Kidney Tumor Registry. we have Many patients with angiomyolipoma have not seen a single instance of true metastasis. 'rhe ·t uberous sclerosis complex, and it is t hought t hat tumors ar·e sometimes mul ticentric, often with symptomatic angiomyolipomas are a forme fruste ti)e· bulk of the tumor located outside the kidney; of tuberous sclerosis. it may recur locally. In one case we have seen a tumor in regional lymph node and in one case ex· Dr. Mostori's Diagnosis: t.ension into the renal vein. ANGlOMYOLIPOMA WITH Plti£DOMl NANT The fact that t he tumo r· is quite large, ex LEIOMYOMATOUS COMPONENT trarenal and highly vascular with tortuous blood ·Histopathologic di<•gnoses subm itted: vessels, frNJuenUy with hemorrhage. may lead · the radiologist to the diagnosis of extrarenal Augiomyo lipom~ , , , . 28 l.ciomyosRr<:omi• , . 20 vascular lesion Pi~ . 3-Crns:; ~~cti on (II kidn~)' and tumor. sclerosis. a substantial number of the clinically reported cases have occurcd in patients without other stigmata or that syndrome. They seem to be, at least on basis of current evidence, more or less benign. I am not aware of any patient who has had a distant metastasis; we think that we have seen one who had a lymph node metastasis. Whether or not this is really a metastasis or represents multicentric origin is uncertain. The practical importance of making a distinction bet· ween this and a renal cell carcinoma or some other malignant tumor of the kidney rests on the possibility of bilaterality, which was well il· lustrated in this case. Since these lesions are benign, one does not want to end up taking out both kidneys. In clinical Dr. Mostofi: J am happy to note that the mn· presentation, it is evident that a. number of these jority of pathologists ugreed with the diagnosis of patients present with a massive hemorrhage into angiomyolipoma. The diagnosis of leiomyosar· the lesion; on radiographic investigation. they are coma is suggested, but the presence of the fat found to have a large tumor that is quite vascular, component, mature fat cells. and the vascular and the problem of what to do about it arises. In component to which I called attention would have practical terms, it does not seem that anything forced a diagnosis other than leiomyosarcoma. short of nephrectomy would have been very feasi· Although for the most part the tumor consists of small vessel tumors. the diagnosis of rhab domyosarcoma is not justified because we did not see any cross striations. There is no justifica tion fo r a diagnosis of renal cell carcinoma. Dr. dclRegato: Dr. C. Perez·Mesa of Columbia, Missouri. W. Bullock of Los Angeles. and Dr. M. Weili·Bousson qf Strasbourg also made a diagnosis of angiomyolipoma. Dr. A. 0. Severance of San Antonio called it a cellular type leiomyoma; he commented that he saw no mitotic activity but that on the variation of size and shape of cells, one might entertain a considera· Lion of malignancy. Dr. David Dahlin of Rochester, Minnesota and Dr. Yvon LeGal of Strusbourg as well as Dr. L. M. Franks of London made a diagnosis of leiomyosarcoma. Dr. Franks commented that he had considered neurofibrosar· coma but thought that the giant cell areas are more typical of leiomyosarcoma. Dr. P. Walter of Strasbourg also questioned the possibility of malignant Schwannoma. Subsequent History: Two days after operation, tho patient died in cardiac arrest. Autopsy reveal· ed the presence of another tumor measuring 7 x 10 em and of the same character in the upper pole of the right kidney and occupying two-thirds of the parenchyma: there were areas of hemor· rhage and necrosis (Fig. 7). Dr. Whitmore: Although these tumors are commonly suspected in patients who have tubular blc, even had one reeogniwd in advance the possibility that it was an angiomyolipoma. T he radiologists are becoming more and more aware of this diagnosis and perhaps can provide us with more and more help in distinguishing these presumably benign lesions from malignant ones. I would like to nsk Dr . Elkin if he thinks that a diagnosis of angiomyolipoma can be made radiographically. Or. Elkin: Not really. Dr. Viamonte and others have described microaneurysms in these vascular lesions of angiomyolipoma that are supposed to be the hallmark of the diagnosis. but we sec little aneurysms sometimes with a renal cell car· cinoma. In terms of the ''epinephrinated" angiogram. many angiomyolipomas act li ke renal carcinomas. Their vasculnr muscle wall is not well deve loped, and. just as with renal cell carcinomas, they do not respond to the epinephrine. Therefore, I do not feel very confident in saying in a given case that it is an angiomyolipoma as op· posed to a renal cell ca rcinoma. I would like to know if Dr. Ceballos·Labat or Or. Mostofi could tell us U this actually arose in the kidney or in the renal capsule itself. Or. Mostofi: The majority. H not all. of those that I have seen seem to arise from the kidney itself rather than the ronnl parenchyma. Dr. Elkin: There wns that fu nny vessel that looked like it might be a capsular vessel. Dr. ~l ostofi: It could be primary extrarenal and secondar ily involved the kidney. We have 1-'lg. 7-Auto~) specimen of right kidnl')' with addhionallar,t seen several patients who came in shock because tumor. or massive retroperitoneal hemorr hage. 7. WILMS' TUMOR WITH NEUROGENOUS DIFFERENTIATION Contributed by y ,·on LeGal, ) 1.0 .. Strasbourg, France The patient was a l·year·old baby girl in J uly l n the left retrograde pyelogram, the calyces 1959 when a tumefaction of the abdomen was are dilated and deformed with no fill ing of the noted. On examination a mass 4 x 7 em was felt in lower calyces; the mass appears to be of the the left hypochondrium. There was no fever and kid ney and may cross tho mid·line (Fig. 2). no apparent tenderness to palpation. I will not consider neuroblastoma, although in Dr. Elkin: There is a large mass in the left favor of this diagnosis is the presence of calcifica· fl ank displacing stomach, small bowel. tr ansverse Lion, but the mass in this patient is intrarenal. colon and descending colon. The lateral border of Wilms' tumor should be the first diagnosis for a the descending colon is irregular; is this feces or solid renal mass in a one year old child. Calcifica· involvement by the mass? The left psoas line is tion is uncommon in Wilms· but can occur and be obliterated. Within the mass is a cluster of faint visualized roentgenologically in about 5 to 10% of calcificaLions (Fig. 1). the patients. If one makes the diagnosis of Wilms' 22 in the kidney itself. so I would not hav., made that my diagnosis. I thought harmartoma would be a very good diagnosis. rr we think it is an intrarenal tumor, then I would not make that unspecific diagnosis o[ retroperitoneal tumor. Dr. del Rrgat.o: Drs. A. Convers of Miami. !\or· man Rosenthal of Tampa and Tohru Ishikawa of Ci ncinnati also suggested Wilms' tumor. Dr. J. F. Wilson of Milwaukee oHer·ed neuroblastoma. Operative Findi ngs: On July 15, 1959, a left nephrectomy and pa1·tial col~ct.omy were carried out. Dr. Mostofi: The kidney itself shows (;on· siderahle ascending pyelonephritis with many tubules filled with ploymorphonuclear leu· kocytes. The t umor presents solid sheets o[ fairly uniform small undifferentiated cells. They have round or oval nuclei witli rine chromatin pattern and rare nucleolus. There is very little cytoplasm. and the cell borders are indistinct. At the periphery of cell masses. the cells assume an elongated appear ance oriented perpendicularly to the basement membrane. The cell m:tsscs are s<•rarated irom each other by delicate bands of fibrous tissue. Scattered throughout, but mostly in the fibrous scptae, arc fr· cqucnt primitive t ubules and preglorneruli. In Hrens there is a ne urogenic differentiation consisting of glial Pig. t- l.1tr"t' mtt,'"' of (ht: leh nank di:-; Jl liWing the ~<~ l umuch, cui• elements \Fig. 3). on 1uHI small inl(•slint•, tumor in an infant under one year of age. Lhc fashion now is to think of fetal hamarLomn (or con· genital mcsoblastie nephroma). I do not know whether calcirication has been reported in these U think not), but its histology resembles ut.crinc !ibroids. and I should think that calcirieation could occur. A combination of big tumor with calcification in a chi ld brings up t he thought of teratoma of the kidney. This is a very rate tumor in the kidney. so nrre t hat I wi ll not t:onsider it seriously. o,., E lldn '~ l mp rc~si o n: 11 CONGENITAL ~ffiS OBLASTIC NEP IIR O~I A 2) WILMS' TUMOR Radiologic impressions submitted: Wilms' tumor . , . , . .4i Neurobla~ t oma .. 26 Mt>sobllstic: nephroma (hmm.:artomat . , • 06 Reuoperitonea.l tumor 05 Orhers 08 Or. Elkin: :\iost thought it was a Wilms' tumor, and we discussed why that is a good diagnosis. In favor of neuroblastoma is calcifica· lion, which in this lesion does go across the mid· line. However·, it appears to me that t he t umor is They have presented a somewhat different histology, but the basic thi ngs, the common denominator in these tumors has been t he presence of three components: undifferentiated nepbroblastic type of elements, spindle·shaped cells (sometimes myxomatous areas), and epith Dr. l\o(ostoli's [)iagnosis: Subse<(uent History: 'l'he patient died in March 1960, cause unknown. WILMS' TUMOR WITH NEUROGENIC DWJ"gftENTIATION Dr. Whitmore: Dr. Elkin's comment about the Histopathologic diagnoses submitted: calcification going across the midline less fre· Wilms' tumor . . , , • ...... 70 quently in neuroblastoma is an important one. I Mt?.soblastk nephroma . • ...... 08 think we should bear in mind the possibility that Carclno.sarc.oma ...... , , , ...... 0~ Neuroblastoma , .. . ~. , , . , , .. , . . , , . . . . 02 you can have intrarenal neuroblastomas, al· Olhers ...... 05 though it is a relatively r are tumor . Certainly from the standpoint of abdominal palpation. if you Dr. Mostofi: 1 would like to wait to discuss the had a neuroblastoma extending across the differentiation between Wilms' tumor and midline, it would probably (eel substantially dff. mesoblastic nephroma when we t alk about an up· ferent than a Wilms' t umor, which is usually a coming case later in. this Cancer Seminar.! do not balotable mass; although it may reach enormous believe the diagnosis of c;lrcinosarc!)ma is size, it usually r etains some degree of mobility. justified. I have discussed the diagnosis of Even in the absence of mobility, most Wilms' neuroblastoma, which we ruled out by the tumors are relatively easi.ly resectable. presence of tubular structures. We liave had four instances in which we have made the diagnosis of As far as the general treatment approach, one Wilms· t umor: case 1, case 4, case 5 and case 7. t hing vastly different is that 20 or even 10 years 24 ago, you had to operate on these cltildren in bowel resection; it is not commonly necessary surgical emergencies. That was more or less the even i·n large Wilms' tumors, but it is an event attitude fostered by the group in Boston, but now that should be taken into consideration in pre the pace of pre-operative work-up is a l ittle more operative planning. leisurely. We put a sign on the bedudo nonouch" because we think that abdominal palpation is a Dr. U. Azar, Tampa . .FL: I believe this tumor potentially easy way to disseminate these was exported to Tampa, not as a plain case of tumors. A recent study from the European Wilms' tumor, but something very special; I think Cooperative Oncology group has suggested that this is what Dr. LeGal implied when he mentioned pre-operative ra'diation theraM in very large renal cell blastema or an equivalent term. There tumors may be justifiable because it re,duces the are severalareas in the sections that unequivocal prospects of rupturing the tumor during its ly rep!'esent glial .or neuroglial elements. Dr. Ar· removal; the American experience does not quite thur Purdy Stout used to mention that his great bear that theory out. friend, Dr. Pierre Masson, believed there was The second consideration is what to do pre something in the nephrogenic blastema that operatively about size reduction in situations either stimulates or develops into neuroglial where the bulk of the tumor is such that you elements. In the same way, malignant schwan would rather not do surgery primarily. If you do noma may un\lergo metaplastic mesenchymal angiograms in some of these children with very elements; one is neuro-ectoderm, and the other is large tumors, you can be rather dismayed by the mesoderm, two different embryonal layers which degree of displacement of the great vessels, the may stimulate each other. I do think that this is a aorta, and the vena cava; it can·be quite frighten special form of Wilms' tumor with definite ing. You are better off not doing the angiogram neuroglial elements. It is a very unusual sort of because even where there is considerable tumor. d.isplacement, the removal of the tumor is usually not that difficult. In the rare instances in which Dr. del Regato: That is why it came out of the pre-operative therapy is utilized to reduce the teaching file, as Dr. Elkin said. Dr. Mostofi has bulk of the tumor, you can use-either radiation already discussed the matter of differential therapy or Vincristine. Vincristine works just as diagnosis in other congenital but benign lesions. I well, perhaps a little better than, radiation would like to point out that these diagnoses were therapy in these tumors and has other advan not recognized just a few years back, and a great tages as far as long-term adverse effects on the number of such congenital hamartomas. patients. aoenomas, etc. were diagnosed as Wilms' tumors. As a consequence, past statistical treatment We commonly utilize a transverse abdominal results of Wilms' tumors on whatever form of incision. In a child the distance between the ilia.c treatment have benefited from these mis crest and the rib cage is relatively narrow, so it diagnoses. Thus, any r etrospe ~ tive analysis of the has the advan.tage of ample exposure, and it treatment results of Wilms' tumor would require facilitates exposure and exploration of the op· a revision of the histopathology. At a Cancer posite kidney, which is essential. We make a Seminar held in Colorado Springs (Cancer great point of carefully exploring the other Seminar, vol. 4, num. 5, 1972) three consecutive kidney because even in children having cases were presented in which the overwhelming pyelographieally or even angiographically normal majority of participating pathologists made a kidneys, small tumor deposits on the opposite diagn<>sis of Wilms' tumor, and none of the three side are sometimes recogni2ed by exploration; wa.s a-ctually a Wilms' tumor. The congenital this modifies the treatment measurements, of mesoblastic- nephroma is a better term than course. Children should_ always be prepared for hamartoma; this diagnosis is being made m.ore the type of operation necessary., in this case a frequently of late. 8. PLEOMORPHIC LIPOSARCOMA OF THE KIDNEY Contributed by D. Eisert, M.D., and A. A. Effron, ~[.0., Milwaukee, Wisconsin The patient was a 53-year-old woman in not included on the film. The tlftb lumbar September 1976 when she complained of lumbar vertebra looks dense, especially on the left; I and of left liip·pains. On examination a mass could would like to see more views of this vertebra. The be palpated in the left upper abdominal quadrant. changes could be due to degenerative disease, but All laboratory tests were within normal limits. I am not sure. The left kidney is big, ;tnd all the calyces do not opacify. I do not see an amputation Dr. Elkin: Because of tlte lumbar and le-ft hip of the ·calyces on this film; that usually means pains, I looked for bone metastases. The left hip is malignant tumor. There is a large mass in the up- 25 Dr. Elkin; I cannot fight very strongly with renal cell carcinoma; t his could be a markedly hypovascular renal cell carcinom:.L T he vessels that we do see extending beyond the kidney do not look liike the kind of vessels usually seen with a renal cell carcinoma; they s·eem a little more r egular. a li ttle more attenuated. J would rule out transitional <:ell carcinoma. lL would be very unusual to have a transitional cell carcinoma in· vade the renal parenchyma and cause a big mass like this; it is more infiltrative, rather than mass p•·oducing. This lesion appears to be within the kidney, and it is spreading very locally. The vessels are going to the kidney, and there is de.struction of the cor tical outline of the kidney. If this lesion were primarily outside ·of the kidney pushing into it, then we would expect to sec a con tinuity ol' the distor ted but still present renal neph1·ogmm. Therefore, I do not think this is ex· trarenal. We see vessels going through the mass, so that would r ul e out renal cyst. per pole of the left kidney; I see no calcification in Dr. del Rcgaio: Dr. Tohru Ishikawa of Cincin the mass (Fig. 1). nati also suggeSt(>d a metastatic renal tumot·, and Dr. Hermenegildo Ramirez-Jime'nez of Mexico The selective left renal angiogram shows City offered a ganglioneuroma. stretching and probably encasement of the (ew Opcrath•c Findings: On September 27, 1976, a arteries to the upper pole. but the mass is quite left nepnrec.tomy adrenalectomy and splenec· hypovascular. or incidental in terest but of no im· tomy were carried out through a transverse inci portanee to the diagnosis is the pressure on the sion; part of the diaphragm was also removed. A r enal pelvis by a segmental artery. '!'he mass is tumor 19 x 15 x 13 em was apparently cncap· huge with displacement of the aorta to t he right. sulated within the kidney. and the faintly opacified inferior adrenal artery is pushed medially. Of special interest is the coUcc· Pi~ . 2-Scl<·d h·e renal t~.ngio_granl showing r~t1 h er hypont~('_u h\r tion of thin and disc1·ete arteries lateral to the kidney. lateral border of the kidMy, indicating extension of tumor. On this film ther e is no AV shunting or puddling of contrast medium as is seen typically in adenocarcinoma. The left paravertebral line is interrupted, suggesting t hat t he tumor is involv ing the extrarenal r etropertioneal tissue. The base of the right lung looks "dirty." so I wish we had a chest film (fig. 21. This might be a hypovascular adenocarcinoma of the kidney, but tho nature of the vessels would not suggest t his diagnosis. Metastases to the kidney ar e often hypovascular, and this diagnosis is possible here. On the basis of lar ge size and nat ure of these vessels, I would make my first diagnosis sarcoma of t he kidney. Dr. Elkin's Impression; II RENAl, SARCOMA 2) ,\Jf:TASTATIC 'fU)IOR Radiologic impressions submiHed: Huna I cell carcinoma ...... ~ . . . ;l8 Tnlnsitional eell carcinom:t ...... l3 AdrfHial carcinom:\. , . . . . . · ~· · . . . .06 Henal cys.L ...... 05 \Vhatcvcl'! , , ...... · · ~ · · · · - · · , , , 01 Others ...... • ...... 15 demonstrated here is not seen. I do not believe fibrosarcoma i.s justified. Dr. del Regato: Dr. L. R. Kime of El Paso and Or. 0. Dahlin of Rochester, Minnesota also made a diagnosis of liposarcoma. Dr. A. 0. Severance of San Antonio labeled it as poorly dirrerentiated mvxoid type of Stout's; he also admitted that it may be clas,sified as pleomorphic liposarcoma of Enzinger. Dr. Leo Lowbeer of Tulsa called it a rhabdomyosarcoma, and Or. L. M. Franks of Lon· don submitted ganglioneuroma; be commented that on light microscopy, he could not deter mine the nature of the giant-cells and fibers, but be did not think it is malignant. Subsequent History: The patient was last seen Fit:. 3-Spind.lt and Sl('llatt' ~hopt•d «118 (x 100). on February 21, 1977. She is doing well and has been expectorating silk sutures. Or. Mostofi: The cortical portion of the kidney shows compression atrophy, interstitial fibrosis Dr. Whitmore: We have seen bronchopleural and chronic inflammatory cell infiltration. The fistulas follow procedures on the kidney. One of fibrous capsule is thickened. There is a large ex our most notable patients was a man who had a trarenal tumor that shows a variegated ap· perinephric abscess; he ultimately developed a pearance. Spindle and stellate shaped cells, bronchopleural fistula and expectorated urine. mature and immature fa t cells Oipoblastsl, mono The problem is reconstruction of the diaphragm, and multinucleated giant and signet ring cells are especially if a lot of it is taken out. seen in a myxomatous background with many delicate branching and nnnstomosing plexiform What strikes me about this patient is that this vascular spaces (Figs. 3 and 4). Mitotic fi gures are lesion apparently began in the kidney. I would occasionally seen. like to ask Or. Mostofi if this is a true liposa~coma of the kidney, rather than a retroperitoneal 'rhe increased vascularity that liposarcomas liposarcoma involving the kidney; the latter is the show and tho unusual intrarenal location of this more common presentation in our own ex· tumor readily explain the radiological perience. Surgical excision is agreed to be the op· misdiagnosis of renal cell carcinoma. Histological timal therapy for these tumors. There is good ly, the tumor is typically liposarcoma with many evidence that radiation therapy does favorably lipoblasts. The presence of the latter exclu~es the affect lesions that are non-resectable, and there is diagnosis of other types of sarcomas wh1ch can good evidence that chemotherapy, including mimic liposarcoma. These tumors are usually regimens of Vincristine and Cytoxan. will have a retroperitoneal with sec~ndary involvement .or salutary effect on non-resectable lesions. These the kidney. Whether th1s tumor was truly ln modalities might best be used in a situation such trarenal, or this impression was formed because as has been described, but it is uncertain, for this of a false encapsulation cannot be determined. is such a rare tumor that no significant ex However, in the slide available for examination, perience is going to be developed in the lifetime the tumor is definitely extrarenal. of any one individual. The scarcity of renal liposarcomas makes unreliable any statement relative tc their Or. J. ~[.Dell, Gainesville, FL: I would like to behavior. There is a report of survival of up to 13 ask Dr. Elkin or Dr. Whitmore to comment on years when death occurred from metastases. Fig. 1- :l:lono and multinucleated tJant and si(nel,«IIJ:I (Jt;;O}. Dr. Mostofi's Diagnosis: ~ ~ ,.,.. j " '\; • I 'f .- LIPOSARCOMA. PLEOMORPHIC Histopathologic diagnoses submiUcd: l - ~,;~ ·..~· ~ ' • ~.A ~ ··-; ~ Li.posanc>1na . , ...... 72 Sarcotnas (rhab 9. RETROPERITONEAL FIBROSIS Contributed by A. F. Lalli, ~l.D. and W. A. Hawk, M.D., Cleveland, Ohio The patient was a 70-year-old man in April l976 Dr. Elkin: Tbere is a left hydronephrosis when he was found to have a pulsatile mass in the associated with a long zone of narrowing of the abdomen. The ser um creatinine was 3 mgm %. ureter at the level of the third lumbar interspace 2S aneurysm, we might have cxr>cctcd the surgeon to have done a prosthesis. I t hink t hat the opern· lio n was a biopsy. This could repr esent retroperitoneal fibrosis unrelated to the aortic aneurysm; that's possible. The bone above the right acetabulum still bot hers me. Then there is t he suggestion of an ex· Ira soft t issue line crossing t he left psoas on the pre-operative fil m. I am thinking of a tumor such as: (a) metastatic tumor to the left retroperit oneal tissue with .encasement of the ureter (e.g. lym phoma). or (b) metasr.asis to the urt•tcral wall. or (c) primary retroperitoneal tumor like a fib rosar coma. However . primary retroperitoneal t umor usually deviates the ureter to a g reawr extent, and we should expect to see tumor vessels. Dr Elkin's lmpres.~i o n: 1) RETROPERTION"EAL LY) LPIJOMA 2) RETROPERITONEAL FIBROSIS Radiologic im pressi ons submi ltcd: RetrQperitoneal fibrosis , 25 Aorticaneurysm , , . 2•1 Retroperitoneal tumors 23 TrJnsiti()nal cell cal'cim>rtltl 07 Carcinon~a ol thu unJLur . oc Others . 08 Dr. Elkin: Retroperitoneal [ibrosis is a fine diagnosis . Aor tic am>urysm i ~ obvious. Pmbably some t hought that u diagnosis of •·etrope•·itoneal tumor was too. easy for t his type of confcl"t)OCe; from there the suggestion of tumor, such us lym· to L5 fo•· a distnnce of about 7 em. and t he ureter phoma or maybe a pr imary ret roperiw neal ~um o r may be made. I do not t hil1k t hat we can consider is displaced a bit later ally. ! believe. T here i ~ t he SUI:(gcstion of un extra soft tissue density line a t ransitional cell carcinoma very ser iously crossing t ho left pso:as line in t he mid lumbar because the urewric nfi rrowing is smooth: the area. i.e. a soft tissue mass. T he bo ne detail of the ureter appears r igid as f<1 r us we can tell in t hat rig ht ilium just above the acetabulum looks ir· film, and t he lumen is quite smooth. I woul d be rcgulr•r ; is this feces in the colon. or is t he bone very sur prised if t his were a necrotic lesion or the abnormal'? !Fig. I) ureter; the same mig ht be said fo r carcinoma of t he ureter unless one was thinking of some kind In the aor togram there arc multiple surgical of peculiar sarcoma!o us lesion of the ureter wall. metallic sutures. There is a fusiform nneurysm of f.n the end, l agree with the majority. the abdominal aorta in its usual location. below fig. :!-Appnn:-nt :ilow ptd u3ion of tht> ldt N"nlll arlf"r) and the renal arteries. Note the good nephrogram and fusifonn antUf)hm tJf tb<" aorta. pyelogram on the right. The left renal artery is still opacified with poor and apparently slow per fusion of the left kidney. and there is no evidence of excretion from the left kidney. The left psoas line now looks irregular . I wonder whether the surgeons were pokin!( around in that region (fig. 21. If we try to link up t he most striking findings. ureteral nnrrow ing and rigidity as well as aor tic aneurysm. t he tirst diagnosis would be fibrosis around t he Mrta. possibly d ue to some leakage of blood [rom t he aneurysm, the ureter be ing encas ed in the fi brosis. I.e. n type of retroper itoneal iibrosis. There is nothing wrong with that diagnosis. alt houg h iL seems too obvious for a ses sion like this. rr t his wore a leaking aortic gastrointestinal tract. In fact, we did a mucicar mine stain to rule this out. In this patient we have the history of ab· domina) aneurysm with leakage, and we are led to the conclusion that retroperitoneal fibrosis was due to leakage of blood. Retroperitoneal fibrosis is more frequent in men than in women. The age ranges from 17 to 83 years with an average of 53 years. The patient may come with abdominal or flank pain, mass, uremia secondary to ureteral obstruction. Ureteral obstruction is usually bilateral. Retroperitoneal fibrosis may be associated with mediastinal fibrosis. aortitis and sclerosing cholangitis. Many etiologic agents have been incriminated, Fig. :l- Rands M hyalinized fibrous lissuc\ nntture- (at nnd such as leakage of blood from the aorta (as in this ''Mtular spnc.:c.•:!J (xl50). case), trauma, infections, drugs, lymphatic and venous obstruction, allergy, etc. Dr. del Rcgato: Drs. Alberto Convers of Miamj and M. W. Elson of Milwaukee also suggested Dr. Mostofi's Diagnosis: compression of the ureter by a retroperitoneal RETROPERITONEAL FIBROSIS lymphoma. Or. Tohru Ishikawa of Cincinnati Hist.opathologic diagnosis submitted: preferred a seminoma. Retroperitoneal fibrosis ...... 51 InOammatory pseudo·tumor ...... 12 Operati ve Findings: On April 17, 1976, a Amyloid ...... , ...... , ... 05 surgical by·pass of the abdominal aorta was done. Pas de diagnostic propose! •...... • . . • . 01 The· ureter was freed of a retroperitoneal mass, Others ...... , ...... 11 which was biopsied: aggregate fragments measured 12 x 10 x 6 em. Fragments were tan· Dr.llfostofi: I am happy to note that almost all yellow in color with hemorrhagic edges and of the participating histologists made the diagnosis r.ubbery consistency. of retroperitoneal fibrosis; inflammatory pseudo· tumor belongs to the same group. I can under· Dr. Mostofi: The section shows two pieces ·of stand t.he diagnosis of amyloid due to polarized tissue consisting of bands of hyalinized fibrous areas, but it was. not amyloid. My diagnosis was tissue. mature fOlt and vascular spaces (Fig. 3.). retroperitoneal fibrosis not due to leak in the aor· There is diffuse infiltration, sometimes intense lie aneutysm,·but the ertdojiathic one. with plasma cells and lymphocytes. Focal polymorphonuclear leukocytic infiltration is also Dr. del Regttto: Dr. P. A. Gikas of Ann Arbor, present. One of the pieces is more hyalini~ed and Dr. F. Moatamed of Salt Lake, and Dr. H. A. Azar has less inflammatory cell infiltration, but these of Tampa also made a diagnosis of retroperitoneal are all still present. The vessels sometimes show fibrosis. Dr. F. M. Enzinger of Washington, D.C. subintimal edema and hyalinization of the wall. designated it a& Ormand's disease. Dr. L. 1\>l. No evidenc¢ of old or recent hemorrhage is seen. Franks of London su~gested aneurysmal wall. The diagnosis of retroperitoneal fibrosis is Subsequent History: The patient was last seen in strongly suggested. However, before this October 1976 and was doing well; his serum diagnosis is made, we must rule out a variety ·of creatinine wa? 1.7mg %. lesions, e.g., lymphoma and metastatic secondary carcinoma, inflammation due to diverticulitis, Ur. Whitmore: If we consider this a case of pancreatitis, and Takayasu's arteritis, all of retroperitoneal fibrosis, and that seems to be the which result in retroperitoneal fibrosis. consensus, I would have to say that it is a very The s,cattered and focal distribution of lym early case from the urologist's standpoint. The phocytes in a .sclerosed stroma raises the ques· fact that the patient has apparently improved tion of · sclerosing lymphoma. However, the with surgery would suggest that this diagnosis cellular infiltrate. consists of an admi.xture of lym· did obtain. phocytes, p·lasma cel.ls and mononuclear cells, and I am a litlle puzzled by the treatment ad· occasionally polymorphonuclear leukocytes ministered. In New York what we usually do for which indicates not only chronic but some Olcute retroperitoneal fibrosis is to dissect the ureter inflammatory process. A second p.ossibility, from the fibrosis, leaving the fibrosis .behind. which should a·lways be excluded in such cases, From the Cleveland Clinic report, I gather that is metastatic carcinoma. often from the they excised a substantial mass of this tissue. I 30 presume that this might have been an encap· pounds. so there is still a substanlial number of sulated tumor. patients with retroperitoneal fibrosis in which the etiology is obscure. Dr. Mostofi has pointed As far as the etiology of retroperitoneal out that sometimes in patients diagnosed as hav· fibrosis is concerned. I am sure you all are aware ing retroperitoneal fibrosis. a careful exami,na· that most cases are not explained. A few years tion of the biopsy material has disclosed a nest of ago. it was observed that a derivative of a drug cancer ceUs in the retroperitoneum; this is cer· used in the treatment of migraine and other tainly well documented now and suggests the headaches was associated with an apparently possibility that desmoplastic reaction to a diffuse noticeable increase in this disease. Many patients neoplastic infiltrate in the retroperitoneum may in whom retroperitoneal fibrosis occurred had no be responsible for this syndrome in some history of injection of any of the suspected com· patients. 10. MALIGNANT LYMPHOMA OF RETROPERITONEAL SPACE Contributed by F. I. Andres, M.D., H. J. Conlon, M.D., )1. Elson. M.D. and J. F. Wilson, M.D., Milwaukee, Wisconsin The patient was a 72·year-old man in May 1976 possibilities: First, amyloid of the ureter usually when he was hospitalized for urinary retention. involves the wall of short segments of the ureter: He gave a history of suprapubic prostatectomy however, it is most frequent in the lower segment two months previously, which had been followed of the ureter. Second, I am intrigued by the by estrogen therapy. The SMA·12 and the serum localized gas-containing loops of small bowel. acid phosphatase were normal. Could this be ureteral involvement £rom bowel disease, such as Crohn's disease. or can the bowel Dr. Elkin: In retrograde pyelography, there is be involved by the same process that is in the wall a tap&red narrowing of the right ureter just of the ureter, such as lymphoma'/ Third, above the wing of the ilium, a narrowing of about metastasis of the wall of the ut·eter; the most com· 2.5 to a em in length; close to the ureteral narroW· mon primaries are stomach, prostate, lung, lym· ing is a peculiar gas containing but not dilated phoma and colon. The patient did have a loop of small bowel. The renal pelvis is markedly prostatectomy followed by estrogen therapy. and dilated, and the contrast medium apparently got he could now l!ave a metastasis to the wall of the through the ureteral stricture easily enough to ureter producing the stricture; lhe primary might ftll the renal pelvis, in fact with enough pressure be prostate. to produce intrarenal reflux. Yet, despite the marked hydronephrosis above the stricture, the Dr. Elkin's Impression: ureter proximal to the stricture is not dilated 1) METASTASIS TO URETER significantly. Note that at the lower end of the 2) DIRECT INVASION OF URETER BY stricture. the filled ureter ends sharply and RETROPERITONEAL TUMOR obliquely as though there is something growing in the medial wall (Fig. 1). Rad iologic impressions submitted: The second film is an intravenous urography. Retroperitoneal tumor . . , , ...... 31 Tr>1.nsitional cell careinorna . . , . . ,, , . . 16 The patient apparently has had a lym· Carcinoma of the ureter .. . . , . . , , . , . . I J phangiography since the last study. I am not Adenocar<::inomlt of prostate. ,...... 09 struck with any grossly abnormal nodes among Inflammatory stric:.tur~ ...... 09 those opacified. The right kidney has a Others ...... 12 nephrogram but no pyelogram. The right kidney Dr. Elkin: I would not put retroperitoneal does not look big. The psoas lines look normal. tumor as my first diagnosis, but I think that it is a There is a collection of gas-containing, nondilated possibility. I do nol think transitional cell car· small bowel loops on the right side. The left cinoma is a very viable diagnosis because this kidney looks nor mal. and there is columnization of does not present as a mucosal lesion; it appears to the left ureter, which makes me wonder about the be extramucosal. I would say the same thing for a lowest segment of the ureter; however, I do not carcinoma of the ureter. Rega'rding adenocar· see it weU enough to say anything about it, so I cinoma of the prostate, what is meant is that the will pass it by (Fig. 2). patient had adenocarcinoma that may have The ureter is narrowed, and it looks like a wall metastasized to the ureter; I think that is a lesion of the ureter. I will mention some possibility. Inflammatory stricture would mean 81 vessels, destruction o[ smooth muscle or renal pelvis and intiitration or renal p:~renchyma. The clinical presentation with ureteral stric ture and the pathological finding of nodular m:tss in retroperitoneum compressing the ureter are not those of pyelonephritis. The cellular in!iltrate is uniform: there is destruction of kiclney and ad· jacent t.issue a$ well as formation of nodules. These are features of lymphoma. In this instance the tumor was apparenLiy retroperitoneal with secondary involvement of the kidney and ureter. Lymphomatous involve· ment of the kidney may consist or dit'fusc enlarge· men! or nodular masses. The former is more fiften seen in leukcm ic situations. There is usually hemorrhage and necrosis. Or. Mostofi" s Diagnosis: MALIG:XANT L ntPIIOMA. i\ODUT.:\R, I,YMPHOCYTIC TYPe Histopathologic diagnoses submitted: ~latignam lymphoma . 68 Chronic Jymphalic leukcrni3 . . . . .03 P~·ctonephritis . . , , . . US Oth"s ...... • 06 Dr. MostoH: The majority or the pathologists came up with a diagnosis of malignant lymphoma or of chronic lymphatic leukemia; I think t hat malignant lymphoma is the correct dingnosis. not having any informacion about the blood count and so on. I think that pyelonephritis can he quite severe sometimes and may even reach the point where follicle formation might be seen. but the Fig. 1-R••HnJ:rAM preln~raph.) sho-.·in~ lnpt-rt•d nnno"inx ..1 nature of cells and the massive diffuse infiltrate riKhl un·ltt whh dilatation or lhl" renal p<'l,·is. are not compatible with the diagnosis of pyelonephritis. there is a retroperitoneal fibrosis kind of thing; it is possible. but I would think this would be less t•t~r. 2-lnlra''t'"nou.s ur: .,graph~· failing lft 'ihow rh::hl n.'Ral likely. pc-hls. Or. del !legato: Dr. B. Weiss of Dunedin sug· gested a car cinoma of the ureter. Dr. J. F. Her· nand c~ of Mexico City offered ureteral invasion by r etropel'itoncal tumor as an alternative. Operative Findings: On June 10, 1976, a right nephro-urcterectomy was carried out. A nodular tumor just beneath the right kidney appeared to enclose the ureter. A 3 em spherical mass at the hilum of the kidney surrounded the renal artery. The kidney wus hemorrhagic. Dr. Mostoli: The kidney and perirenal fat are infiltrated with lymphocytes. In areas the cell in· filtration is dense. somethimes with follicle for· mation (Fig. 3). In others the c.ells are separated by fibrous tissue. For the most part, the cells are mature lymphocyws with many irregularly shaped coils. There is infiltration of walls of the bone marrow aspiration prior to the initiation of radiation therapy, but this showed no evidence of infiltration by lymphoma, no evidence of leukemia, and no evidence of adenocarcinoma of the prostate, which in fact this patient did have. His death was due to pelvic obstruction of the remaining ureter by a moderately well· differentiate (I adenocarci noma; the patient refus· cd a urinary diversion procedure. Dr. delRegato: When Dr. Mostofi says that on that basis of ''not having information about the blood," this is a malignant lymphoma, be means that a simple blood count can destroy all of the morphologic assurances. At autopsy of chronic lymphatic leukemias, the kidneys are often in· iiltrated. It is also obvious that some of these pa· 1-'lg. :'-Ot'nse in£iltratiun with lym phnC::)' lCS and folli<'l(' tormu· tients never have a peripheral blood count that is lion. necessarily diagnostic. It is our opinion that there are formes frustes of chronic lymphogenous Dr. del Regato: Dr. L. J. Clown• of Milwaukee leukemia, undiagnosable in lymph nodes, often also made a diagnosis of poorly differentiated mistaken for lymphosarcomas. malignant ly mphoma: Dr. R. L. Font of Washington. D.C. also called it nodular. Dr. L. M. Or. M. Vuksanovie, Miami, FL: Dr. Whitmore, Franks of London made a diagnosis of chronic would you be kind enough to comment on lymphatic leukemia; he observed abnormal white estrogens as a routine procedure in apparently cells in the blood vessels. Dr. A. L. Vargas of El localized adenocarcinoma of the prostate. Also, is Paso preferred pseudolymphoma. Dr. P. A. Gikas orchiectomy indicated in treatment of this of Ann Arbor saw only chronic interstitial disease? nephritis and peripelvlc urine granuloma. Dr. Whitmore: Current evidence indicates Subsequent History: Following ope•ation. the that endocrine therapy of breast cancer and patient received radiotherapy through large estrogen therapy for carcinoma of the prostate fields 18 x 23 em in diameter, receiving a total fall into the same category. There is no survival dose of 3,500 R in 35 days at the midplane of the advantage in initiating treatment early; it is bet· upper abdomen on the right side. In January ter to wait until there is a compelling indication 1977, the patient presented hematuria and on before initiating treatment. Furthermore, in view January 23, 1977, he expired of uremia. No autop· of the Veterans Administration study, evidence is sy was done. now accepte d that estrogen therapy may have an adverse effect on the incidence of thromboem· Dr. Whitmore: At autopsy lymphomas and bolic phenomena. particularly when estrogens are leukemias involve the kidn ey 1>robably over half used in a dose of 5 mg or greater daily. For about the time. In clinical terms the type of presenta· 15 years. we have not utilized endocrine therapy lion demonstrated here would be very rare (less in the treatment of localized carcinoma of the pro· than 5% ). This is not to say that there may not be state. ureteral obstruction from lymphomatous masses arising from adjacent lymph nodes, but it would As far as the relative advantages of estrogen have to be regarded as very rare. When there is a versus castration are concerned. I think it is fair lesion that does not fit any particular pattern, the to say that t here is no convincing evidence of the possibility of lymphoma ougM to be borne in superiority of either method. They arc about mind; every effort should be made pre·OP· equal in their effectiveness, and patients who eratively to rule out th11t diagnostic possibility. have had a good remission with estrogens do not Not only did this patient have an operation that get a second remission from castration, just as pn· might have been avoided. but also he might have tients who have had a good remission from castra· lived a little longer rather than dying of uremlu Lion do not get a second remission from estrogen. had that other kidney still been there. There are facts from clinical experience that sug· gcst that these methods may supplement each Dr. F. Wilson. Milwau kee. WI: At Dr. del other. Rcgato's request, I reviewed the situation before It has been well demonstrated that doses of Lhe Cancer Seminar. The patient never bad an ab· estrogen on the order of I mg daily may not pro· normality of the peripheral blood except for duce in every individual the physiologic change anemia from loss of blood. There was never an ab· responsible for beneficial effect, that is, a phar· normality of the white elements. He did have a macologic castration. In most individuals 1 mg n 33 day will depress pituitary gonadotropins and pituitary gonadotropins rise to normal levels; ac· result in a pharmacologic castration, but there tually gonadal function will return too, and an· are a few individuals in whom 1 gm a day will np· drogens will come back up to normal. parenlly not do this. Tbeir androgens can be Let's say you take a patient with carcinoma o[ measured to find that there is only a transient the prostate and with a pain in his right hip. a big, suppression or none at all: in such individuals. a hard nodular prostate, and an elevated serum dose or 8 mg has invariably resulted in a phar· acid phosphatase. You place him on estrogen or macologic castration. There is some evidence in castrate him, and he has a remission. His acid animal systems that estrogen may have a direct phosphatase falls, the pain in his right hip disap· cy totox ic effect on the prostate or that it may pears, his prostate becomes small er and softer. somehow interfere with some mechanisms, hav· He may fit into a certain group or patients, even ing a salutary effect on the physiology and though you continue estrogen therapy: he may histology of the prostate. However. this has not have pain in his left shoulder whun he relapses, been documented in man: it is a hypothesis that but the pain in the right hip may never come remains to be fully explored. Sometimes large back; his prostate may never grow again, and his doses of diethylstilbestrol will apparently pro· acid phosphatase may never rise again. Yet, he duce symptomatic remissions in patients who dies of cancer of the prostate. What this suggests have relapsed following conventional dosages of is that either the original population or prostatic estrogen or castration, but the mechanism of the cancer cells has undergone an adaptive change to effect is obscure: since it bas not been associated· the endocrine manipulation, or there has been an with any clear cut objective evidence of benefit. initial multiple clone population of prostatic one must keep an open mind as to what is cancer cells, some of which were responsive to en· happening. doerine manipulation and some of which were not. Dr. P. Berger, Dunedin, FL: There is an article Let's go back to your original question. In the published in Seminars on Oncology giving the patient who was castrated and then placed on results of a cooperative study of various cytotoxic chemotherapy, the castration continues to sup· agents in metastatic carcinoma of the prostate: press that original clone of cells, the endocrine the article purported to show that both 5 FU and responsive cells, so that the tumor never Cytoxan had statistically significant effects. relapses. However, he gets another clone of cells There is one cryptic statement made in the article that is active; we then treat it with chemotherapy that intrigued me, that in spite of the statistically and get a certain response. In the analogous significant value of the drugs. not a single population of patients ~r eat ed with estrogens, we response was seen in any patient who had not had stop the estrogen therapy before starting a previous orchiectomy. chemotherapy, and both clones of cells could become active. I am not s\U"e ~hat is the explana· Or. Whitmore: I am aware of that observation. tion. but it is the only one I have been able to put but I am not sure what the significance of it is. I together. In subsequent reports of that study, ean think of one reason that might explain it: I that point has not been mentioned again: I am not believe the patients who had been treated by sure but what it was an artifact. When patients estrogens were taken off the estrogen therapy with carcinoma of the prostate who have bad some weeks in advance of the administration of estrogen therapy are subsequently castrat.ed, the cytotoxic agents. Obviously, the patient who they often have relief from pain for a month or has been castrated has had his endocrine therapy, two, but the probability is that this is a conse· and it is permanent. With regard to the overall quence of the stress response to the operation physiology of a patient who is on estrogen itself, that is, an adrenal steroid response to the therapy and ht•s it discontinued, you would ex· stress of the s.urgical procedure. I suspect that if peel that the effects would disappear. After the these pati'ents were told they were going to have estrogen therapy has been stopped, gonadotropin an orchiectomy, most of them would get the same function returns over a period of time, and stress response, given enough time. 11. CONGENITAL MESOBLASTIC NEPHROMA J. Durham, ll.D. and D. Mc)'Crs, M.D .• Columbia, Missouri The patient was a 3·month-old baby girl in blood level of r enin was remarkably elevated, and November 1975 when she was admitted because there was also hypoglycemia. of projectile vomiting. On examination a mass could be palpated from the right nank to the Dr. Elkin: There is a big soft tissue mass occu· midline. The blood pressure was 240/140: the pying the entire right abdomen and extending 34 quick search of the literature. In 1968 Nissan et al reported that up to that date, about 100 cases had appeared in the literature of patient·s with hypoglycemia associated with extrapancreatic tumors. In most, the tumor was of mesodermal origin and usually located in the thoracic or retroperitoneal area. Of the mesodermal tumors, fibrosarcoma was the most common. Yet, there ha v.e also been reports of hypoglycemia associated with tumors of epithelial origin, most commonly hepatoma and adrenal carcinoma. Most of the tumors producing hypoglycemia were malignant and very large. I did not come across any case of Wilms' tumor in the group; there is at least one neuroblastoma. The mechanism of pro· duction o[ the hypoglycemia is not clear, the sug gested possibilities including: a) insulin or insulin like material secreted by the neoplasm, b) pan· creatic stimulation by the tumor, c) suppression of insulin antagonists, dl excess glucose consump tion by the large neoplasm. Consideration of Wilms' tumor under the age of 1 year should bring up the consideration of a fetal hamartoma. I know of no reports of hypertension with fetal hamartoma, but I should think that zones of isclremia of what renal tissue there is Ji'ig. 1-Large soft til;~uc mass: of t-he righl side of the ubdom<'n could lead to increased production of renin and pressing down on lhl~ blnddcr. hypertension. However, the presence of hypoglycemia would favor the diagnosis of a across the midline as demonstrated by lateral malignanL tumor. displacement of the left ureter.! see no excretion from the right kidney, arrd there is no calcification in the mass. The smoothly bordered mass is This mass could be fluid-filled, i.e. a huge pressing on the dome of the bladder (Fig. 1 and 21. hydronephrosis. The smooth contour of the mass would favor this. There are reported instances of I believe that the mass is in or replaces the hypertension due to hydronephrosis. I see no right kidney. Any renal ma·ss can, on occasion, crescents or co.iltrast medium or no rims of re result in increased elaboration of renin with con maining renal parenchyma to aid in this sequent hypertension, apparently by interfering with renal blood flow; zones of renal ischemia Fig. 2-M.as.~ appt>ars to repln<:e the righl kidney. then lead to the increased renin production and the hypertension. Hypertension has been described not infre quently with Wilms' tumor, apparenlly on the basis of pressure of the mass on the kidney. In such cases; the blood pressure comes back to nor mal after removal of the neoplasm or sometimes after radiation therapy. In addition, there are reports of alleged secretion of renin by the Wilms' tumor in which case the hypertension is cured or improved after removal of the tumor but recurs with the occurrence of metastases. Also, increased renin content has been shown in some of these tumors. Usually, the hypertension in Wilms' tumor is noL so high as the 240/140 of this patient, but le.vels as high as this have been reported. How ·about the hypoglycemia'? There are tumors which have inappropriate secretion of en docrines, and I am suggesting Lhat this is a Wilms' tumor secreting renin and possibly in sulin. The hypoglycemia puzzled me, and I made a diagnosis. Also, hydronephrosis could npt account Adrenal e~r<:i n oma . . ,. , , ...... 09 for the hypoglycemia. Others . ... . •.. •...... - .II There have been reports of a few cases of Dr. l,lkin: Having said that this is an intra secreting tumors of the i.uxtaglomerulat Neurobl.astoma...... 4 ~ •• • •• • ~ . 39 Wilms' t\Jmor...... 21 Pheochr<>m<.)<:yL<· ,ma . . . .. _ . .. , . . . • . . .. 13 Operative Findings: On February 18th, 1976 a laparotomy was done. A huge apparently encap· Pig. a- Large h(:mutrhagie. parlhllb· <:r~lic. ntnss. "Xo kidn(•r sulat 36 <~ , .. tissue and raise the question of a possible associated Wilms' tumor. To rule this out, it is ab solutely essential to study the tumor extensively. We have seen several cases in which some areas of the tumor were typically mesoblastic nephroma; in other areas, there was either a definite undifferentiated sarcoma or an un mistakable Wilms' tumor. Thus, we have come to regard this group of tumors as constituting a spectrum of nephroblastic neoplasms ranging from benign mesoblastic nephroma at one ex treme to Wilms' tumor at the other. It is therefore imperative that many sections be taken from such tumors. Hypertension is not in frequently seen in Wilms' tumor (as it is also seen in association with other kidney tumors). This and other clinical findings ~£ Wilms' tumor may be present in mesoblastoc nephroma except for hemihyper trophy and/or aniridia, which have not been reported in mesoblastic nephroma. Dr. Mostofi's Diagnosis: CO:-IGENITAL MESOBLASTIC NEPHROMA HislopatholoJ,.;c diagnoses submi tted: Congenital mesoblastie nephroma. ,, , ,1lt (Doland'!;, Bolandi's & Jande's. ctcJ Wilms' tumor . . . ~ ...... , ...... 23 Fig. 4-I~ I Uhi ur hnlnt\lurc nt'l)lmHijj and tubules with Other benign...... 15 O\'t•rgrowth of ,\ pindlt.... s hapt:d ..:tllg (x1IO) . Dr. Mnstofi: I want to call attention to the facl the entire side of the :~bdom e n and extended to that a number of t hese cases we called the left. It was completely removed (Fig. 3). leiomyomas, hamar tomas, leiomyomatous hamar tomas, etc. about six or eight years ago. Dr. Dr. Mnstoli: Islets of immature nephrons con Bolande offered the term mesoblastic nephroma. sisting of fetal glomeruli and tubules are seen in A_ few years back, we wrote a paper in which we terdigitating with massive overgrowth of in doscussed 20 cases of this condi~ion, many of terlacing bands of spindle-shaped cells (Fig. 4). whoch had been sent in as a Wilms' tumor, or sar For the most part, the nuclei arc more or less coma of various types; we demonstrated that cigar-shaped; the cells are fairly uniform and they all survived, that this lesion should be mitotic figures are rare. In a fe w areas, mor~ par regarded as benign, and that the patient should ticular_ly in what appears to be the wall of a cyst, not be treated. In Gainesville I was shown a slide there os marked condensation of cells. which are of a case histologically similar to this one that had poorly differentiated, have scant cytoplasm and been diagnosed originally as mesoblastic lack the regular interlacing pattern seen nephroma. The Joeal pathologists made a elsewhere. The tumor appears to extend to the diagnosis of a malignant tumor, and the patient perinephric adipose tissue (Fig. 5). bad received radiotherapy. The slides had been The bulk of the tumor presents the characteristic histology of congenital mesoblastic nephroma (fetal leiomyomatous hamartoma). In addition to primitive nephrons and spindle-cell elements, cysts of varying types and nests of car tilage are often seen. These tumors have often been misdiagnosed as Wilms' and the patients overtreated, sometimes with fatal results, but it has been demonstrated that they arc benign, even those that may s how many mitotic figures and/or extend outside the kidney. Nephrectomy is all t hat needs to be done in such cases. In this pa tient the disturbing features are the areas of un di fferentiated cells that suggest nephroblastic sent t o sever al distinguished pediatric Or. Mostofi made the principal point I would pathologists; one from Missouri got on the have made: that there are sources of error in telephone and said to stop treatment, that this recognition of congenital mesoblastic nephroma. was benign. However, the child died of extensive These are tumors that occur very early in life; as local rec·urrence within nine months. We have the child approaches one year of age, you must be also seen two instances of mesoblastic nephroma more and more suspicious of the diagnosis. The in which a Wilms' tumor could in fact be seen principal source of error is the histologic inter· elsewhere in the section. Whenever we get these pretation that will vary from one pathologist to now, we beg the pathologists to please take addi another. However, perhaps the most important tional sections to make sure that we are not miss consideration is the fact that. increasingly, this ing the Wilms' tumor elsewhere in the section. whole phenomenon of renal blastoma, Wilms' Congenital mesoblastic nephroma is a benign le tumor, and hamartomatous lesions is being sion; complete excision of the tumor is the treat recognized as representing a whole spectrum of ment of choice, but I would like to emphasize that possibilities. The clinical behavior of each of these it is important to take many sections in order to lesions has to be better defined before we are in a r ule out the possibility of a Wilms' tumor. We can position to make any categorical recommcnda· see these tumors as being a spectrum with Wilms' tions. The evidence c.learly suggests that the le· on one end and benign mesoblastic nephroma or sion in this patient that was pointed out by Dr. leiomyomatous tumor on the other. Mosto!i is indicative of a lesion that generally bas a benign presentation and course. Yet, there have Dr. del Reaglo: Or. E. Philippe of Strasbourg been local recurrences and deaths from what and Dr. C. Rich of Cleveland as well as Dr. F. M. were called mesoblastic nephromas. As Dr. Enzinger of Washington, D.C. also made a Mostofi pointed out, something else might have diagnosis of congenital mesoblastic nephroma, been present in the tumor that was not recogniz previously known as fetal hamartoma. Or. E . ed. One must examine these tumors very well Fantelli of Cleveland, Or. Maria Viamonte of pathologically before putting them into any one Miami, and Dr. H. A. Azar of Tampa called it a category; we slill have to say that total ex· Wilms' tumor. Dr. D. Dahlin of Rochester, Min perience of t his lesion is still so small that it nesota submitted a diagnosis of Wilms' tumor would be a little premature to put them into pure with areas like mesoblastic nephroma. Dr. M. ly benign or malignant categories at this stage of Weill·Bousson of Strasbourg offered a diagnosis our knowledge. of tu'.'ror-of'the juxtaglomerular apparatus. . Dr. Henry Azar, Tampa, FL: I would like to of ficially apologi2e fo r my diagnosis of Wilms· Subsequent History: Following operation, the tumor. You realize I seldom see three month old hypertension resolved, and the patien t appeared babies at the Veterans Hospital. Ho\\•ever, there well when seen December 14th, 1976. Dr. J. B. was an area that looked li ke Wilms' tumor. In a Doolittle of Mexico, Missouri examined this pa· situation like this, I wonder whether or not the tient in the first week of March 1977. He reported pathologist should carefully say congenital on the telephone that the child is in excellent mesoblastic nephroma with an area suggestive of health. Wilms· tumor. Dr. Whitmore: Some 35 years ago, an associa Dr. Elkin: There arc some specific clinical tion between hypertension and Wilms' tumor was !indings with this patient that are fairly si$nifi first described: it was pointed out that it could be cant. These clinical findings have been assocoated due possibly to constriction of the renal capsule in the past with Wilms' tumor. Have you seen a or that it could be due· to interference with the mesoblastic nephroma with hypertension and arterial circulation of the kidney. The reap· hypoglycemia? pearance of hypertension following surgical removal of the tumor or with the appearance of metastatic disease has suggested the possibility Dr. ~lostofi: I am sorry that I cannot answer that the tumor itself could be responsible for the that question. We are oow reviewing our production of substances causing the hyperten mesoblastic nephromas or leiomyomatous hamar sion. Recent work has suggested that the tumor tomas to see how many of them we misdiagnosed; may produce either renin or a renin precursor r have already seen three instances of other le· that could be directly responsible for hyperten· sions. In this case, I argued with myself n greolt sion. Unfort unately, children with Wilms' tumors deal over whether or not I should say "with possi· do.not have their blood pressures taken frequent· ble malignant areas," or something like t hat. but I ly because it is hard to do this with an infant: refrained from doing so because I wan~ed to em therefore. there is not as much data in this ar ea as phasize the benign, the identifying recognition of we might expect to have. this group of lesions. 38 Dr. Carlos Perez-Mesa, Columbia, MO: The.re are References Oa"idsQn. J. K.. and Clark, F. F. R.: Ret!in-sccrcting jux· abO!!_t eight or ten papers reporting this type oE tag:lomcrular cell tumor. Brit.. J. R.'ldiol. 47:594·597. 1974. Nrssan, S. • Bar·Mnor. A .. artd Sho.tfrir, E.: HypoglycE!mitt in tumor with hypertension. Because of the large patients with advanced tumors. New _Eng. J. Ml!d. 278:741. amount of renin along with hypertension that was !968. Mitchell, J. D., B(lxter. T. J,, Blair~Wcst, J. R.. and Me· demonst!'.ateg in this case, slides were sent to Credie, 1). A.: Renin h!\• als in nephroblastoma (Wilms' tumor). R~}JQrt of a renin seereting tumor. Arch. Di,s. Child. ·15:376·384. sev~ra! of the authors of these papers, and they 1970. confirmed that it was indeed a case of congenital Silveutein. M. N .. Wakiiu, K. G.. and Bahn. R. C.: Hypoglycemia associated with neo'plasia. Cancer t9: 1274 133, mesoblastic nephroma. l9!ffi. 12. CARCINOSARCOMA (?) OF THE BLADDER Contributed by Joseph Kuzma, M.D., Milwaukee, Wisconsin The patient was a 68-year-old woman in April I think that we are dealing with two separate le 1976 when she complained of hematuria and nyc· sions. In respect to the kidney, the two best bets turia of five weeks duration. The physi~al ex statistically are neoplasm (e.g. renal cell car· amiriation and the laboratory tests failed to cinoma) and cyst. The differential cannot be made reveal any abnormalities. with anY. confidence of the urogram. I would favor the diagnosis of the kidney mass being a large Dr. Elkin: There is a large, apparently cyst, but it could just as well' be renal cell car smooth, round mass in the upper pole of the right cinoma. kidney, pushing on but not invading the adjacent The bladder mass is, I believe, a neoplasm calyces. The mass is relatively radiolucent com rather than blood clot. A blood clot is more apt to pared to the nephrogram of the renal paren be rimmed by contrast material, whereas in this chyma. There is no calcification in the mass. patient the contrast mateFial all collects caudal to There is a large mass in the bladder where the the bladder mass. Therefore, I believe that there contrast material collects along the noor of the is a primary neoplasm of the bladder. Statis· bladder with a lobulated interface between the tically, it should be .transitional cell carcinoma, bladder tumor and the contrast. material. Also and I have no basis for defying these statistics. I present is ·a medial dispjacement of the lower seg· have had no personal .experience with primary ment of the right ureter as well as a suggestion of lymphoma of the bladder, but I am told that streaks of contrast material in the bladder as though between lobules of the mass (Fig. 1). f'ig. 1-Lnrgc mnJ:~s u£ the upper pole or the right kidney nol ap· par\•ntl)' invading the ca.frce.s. If we try to tie the two masses together, we could consider the renal mass a lar.ge primary neoplasm (e.g. renal cell carcinoma) and the blad der mass a big blood clot. However, I do not believe that the bladder mass is a blood clot, so I will rule out this possibility !Yig. 2). Could this be a multicentric occurrence of tran sitional cl)ll carcinoma. i.e., of the renal pelvis with invasion of the parenchyma as well as transi tional cell carcinoma of the bladder? Except for a pressure ·deformity, the calyces appear normal. Also, extension of transitional cell carcinoma of the pelvis into the renal parenchyma does not produce a well defined big mass like this, so I will not consider this possibility. Could this represent a congenital anomaly, such as duplex pelvis with separate ureters, there being hydronephrosis of the upper segment of the pelvis due to ectopic ureterocele? I would say no. I think that there is a complete pelvis present with adequate number of calyces. The bladder fill ing defect of ectopic ureterocele is smooth, not lobulated, and would be surrounded by contrast medium. mononuclear or multinuclear giant cells. The cytoplasm also varies a great deal from eosinophilic to vacuolated. clear and amphophilic. The growth pattern varies too. In some areas the tissue is quite edematous, in others, dense and cohesive. In these areas the cells have nn epithel ial appearance Wig. 4). Elsewhere large cells with bizarre hyperchromatic nuclei are seen and with a large amount of eosinophilic. vacuolated or amphophylic cytoplasm suggesting rhabdomyoblasts (Fig. 5). Mitotic figures. especially abnormal ones. arc frtHlueni. There is focal lymphocytic infiltration. The history and the initial slide we received led us to assume that the slide represented renal tumor and that we were dealing with a malignanat tumor. probably carcinoma or rennl pelvic origin. Alt hough the s lide s howed are this confer ence is the difference in the opinions or •• pathologists. I was wondering if Dr. del Regato could tell us what percentage of the opinions are sent in by practicing pathologists and what arc sent in by urologists. Also. bow does Dr. Whit· more respond to a defensive pathological diagnosis like benign mesoblastic nephromn where there is a spot thut I t hink is a Wilms"? Dr. del Regato: First of all. t he historJathologic diagnoses are rendered by pathologists. Several • years ago at one of these seminars, our guest radiologist, a prominent radiologist from San Francisco, suggested that our tabulations mixed residents in training with professors of radiology and that it wasn't fai r to put t hem all together as • though the opinion of one were equal to t he other. At t he next seminar, we chose six prominent l''ig. 6-Suggcstion ol rhnbclom)'ObhuU II: (x200). r·adiodiagnosticians and marked their diagnoses on the slide with an asterisk in t he column where we have no method for preventing new lesion;, their diagnosis fell; they not only did not do any which are the principle cause of treatment failure better, but they did worse than the residents. As in patients treated conservatively for bladder you know. this is anonymous, and participants are cancer. free to sign or not sign their name to the Dr. Paul Berger, Dunedin, FL: One of the diagnoses; all we know is that they are par· things that I always find most disturbing about ticipants and that they may be residents in train· ing in radiology or in pathology. Some of them fo'lg. 7-Photomitrognph nl slide submitted by contributor with may have been urologists, but very few. eell1 prt-senting tross striation 8-. • 42 Dr. Whitmore: Pathology is, of course, the ing to improve ourselves all the time and to cor· rock on which the whole foundation of our rect our errors, but histopathology remains therapy rests. This is not to say that we essentially the rock on which the whole cancer necessarily regard them as infallible, but if you do therapy is necessarily based. not believe the pathologist, you are on a treadmill Dr. J. Kuzma, Milwaukee, WI: A friend recent· as far as therapy is concerned. ly told a story that befits this ease: an old We see patients referred for various forms of gentleman pathologist was retiring. A t. his therapy, and we get slides for review; many times testimonial, they asked him if he had to do it over we disagree with pathologists on the grading of again, would he do anything differently: he bladder tumors. I think this is in part due to our thought for awhile and said yes. he would spend own system; what we consider papillomas, others less time looking for cross striations. may call grade I papillar y carcinoma. Thus, we ;trc one grade lower than most other pathologic Dr. Mostofi: I am sorr y; I did not know that Dr. gradings. or those patients referred for cystec· Kuzma had sent a slide of cross striations. I would to my , we also see ones with the usual pattern of liked to have seen it. I think that the percentage multifocal grade Ill papillar y carcinoma: we of error can be less when pathologists and review t he slides, and if it looks like a papilloma. urologists work closely together. There are we fulgurate it and send the patient back. techniques available that can be applied in modern day and perhaps decrease the level of What bothers me is the one who has his bladder removed for papilloma somewhere else, and I am mistakes made in path9logy. sure that this happens a great deal. This is a real From the pathological point of view, sta ndar· basic problem in pathology, one that is being ad· dization is much more serious than from the dressed by the World Health Organization and clinical point of view. Some years ago, Sir David the UICC in an effort to get everyone to agree to Thomas of London took a number of slides of blad· call it by the same name and then treat it in a der tumor to several centers in New York and got more appropriate fashion. The same is true of car· diagnoses on the same case ranging from cinoma of the prostate. Within the past two papilloma to papillary carcinoma grade Ill. For 20 weeks, I have seen two patients from California years, I have been working on getting who has been advised to have radical prostutoc· pathologists to agr ee on a grading system of tomy on the basis of histologic diagnosis of pathological diagnosis of urological tumor s as cancer; they came to our institution because they well as other tumors. Maybe in the next 20 years, wanted to have a radioactive implant instead of a l will succeed. radical prostatectomy. We reviewed the slides. and it was atypical benign hyperplasia. It was not References a cancer at all. You wonder how often this hap· Pontius, B.B.. Nourse, M.H .. P••· L.. 'nd McCallum. D.C.: pens around the country. There may be Primary maJigoant lymphomu of the bl.:.ddcr. J. Urol. 9()-.58-61., weaknesses in histopathology because 1963. Santino. A.M., Shumaker, E.J., and Cartes, J.: Primary pathologists are human, like all clinicians. The on· malignant lymphonus of lbe bladder. J. Urol. 10.1:310-313, ly thing that we can say is that we should be try· 1970. 13. TRANSITIONAL CELL CARCINOMA OF THE BLADDER Contributed by Paul Berger, M.D. and W. R. LaRosa, M.D., Dunedin. Florida 'l'he patient was an 84·year-old man in March We are told that cystoscopy showed a mass of 1976 when he complained of hematuria of four the dome of the bladder and right side of the months duration. He gave a history of prostatec· posterior wall. I would have imagined that the tomy in 1965. There were no physical findings. On right side of the anterior wall is also involved cystoscopy there was a mass of the dome of the since we can see the filling defect quite clearly on bladder and right side of the posterior wall. the prone projection. Dr. Elkin: This urogram in prone position Could this be inflammatory? Vesical tuber· reveals Paget's diesase of the pelvis, and there is culosis can result in a small bladder, and tuber· a filling defect of the right lateral portion of the culomas can give masses in the bladder bladder as well as an extensive filling defect of simulating carcinoma. However, caleifieation the dome of the bladder (Fig. 1). hardly ever occurs in bladder tuberculosis, and 43 bladder might be possible, although I do not think so in this case. I discussed tuberculosis of the bladder, and I have r uled tha~ out on the basis of the calcification, among other things, but primari ly c.alcification in the bladder. Paget's disease of bone is there. Dr. del Regato: Dr. R. Byhardt and Dr. A. C. Speranza of Milwaukee offered an impression of transitional cell carcinoma. Dr. A. Convers of Miami preferred infiltrating carcinoma or the bladder in a patient with Paget's disease. Dr. J. F. Wils_on of Milwaukee felt that the picture fitted a Fig. 1- 111111n" dt•rccc or lho right side or tho bluddcr. carcmoma of the prostate and its consequences. usually there would be associated evidence of Operative Findings: On June 24, 1976, a tuber culosis of the ureters or kidneys. I cou ld transurethral resection was carried out: 25 grams mention a handful of types of non-neoplastic of tumor were removed, and the remainder was cystitis like eosinophilic cystitis, cystitis cystica. fulgurated. Thiotepa was instilled. bullous cystitis, chronic interstitial cystitis, but they do not look like this. and they do not con· Dr. Mostofi: The normal mucosa or ~he bladder lain multiple calcifications. The bladder or is replaced by a papillary transitional cell car· schistosomiasis hematobium characteristically cinoma with moderate to marked anaplasia (Fig. has linear calcification in its wall, not the multiple 2). The tumor is superficially infiltrating the projecting calc ifica~ions described here, and I lamina propria. and there is invasion of several would suppose schistosomiasis of the bladder superficial lymphatics as well as blood vessels would be about as common in Dunedin, Florida as !Fig. 31. In areas the tumor shows a glandular it is in Bronx, New York. structure. In one piece the supporting stroma is I believe that we are dealing with a neoplasm. Pig. 2-ltcplacement of bl~;tdder mut ogtt b)' transhi•·ui Dr. I~ Ikin: StaListically, transitional cell car· cinoma is the best diagnosis. Without stipulating the type of course, infiltrating carcinoma is fine. Carcinoma of the prostate extending around the ~~ ~,~~ Yi.-....~ ;"'.:, ·~~, . :t.; ;r- ~ \Y .. !'·' ,.. • ••· I) e ~:4 •l I XIJ • w,; .~ _.d ... • ;- • . ., . :l't9 ' ~ ..... ~ !. ~.tr-',•t,~:~ ~!~,r ._. . 14. LEIOMYOSARCOMA OF THE BLADDER Contributed by .J.D. Cox. M.D. and H. J. Conlan, l\I.D .. Milwaukee, Wisconsin The patient was a 41·year·old woman in Dr. Elkin's Impression: January 1975 when she complained of suprapubic I) ENDO~fETRIOSIS pain. On examination she. appeared pale but in 2) MYOSARCOMA good general condition; there was no palpable mass. Cystoscopy revealed a necrotic mass on the Radiologic impressions submitted: right side of the dome of the bladder. Transit.ional cell carcinoma. ... . 37 Squamous cell tarc:.inoma. . . .. : . . 12 Dr. Elkin: We are presented with an oblique Endometriosis ...... 12 Ad6no¢~r¢inoma ...... 06 projection of a cystogram. The bladder is. . large Sarcoma ,, , ...... · ~ ...... ~ · .06 and filled with over·dense contrast medium. At Mctast.:itic bladder tumor ...... 15 the dome there is a 6 em £illing defect, protruding Other& .... , ...... 12 in smooth fashion for one centimeter beyond the Dr. Elkin: Transil.ional cell carcinoma i:s a projected externat surface of the bladder. The mass protrudes within the baldder lumen by legitimate thought for a bladder lesion. I feel that about 2 em at its greatest depth, and the inner this is a lesion not of the mucosa but of the wall border of the mass might be lobulated. It looks because it is such a smooth tumor, so I would not like a wall lesion to me, i.e. submucosal, at least make that my first diagnosis. I would rule out initially (Fig. 1). squamous cell carcinoma for the same reason, and I think it is apt to be found in an older age group Cystoscopy showed a necrotic mass; if the le· as well as in patients with a history of stones or sion is a wall lesion, it must have penetrated the infection. Adenocarcinoma is going to be discuss· mucosa. 'I am going to suggest that this is en· ed a little later. I do not know what the dometriosis involving'the bladder wall. There~re radiological findings of adenocarcinoma might be. no characteristic radiological findings for this I think sarcoma would be a wall lesion, and that diagnosis, but a more detailed history would help. would be what I would go along with as well as en· It could be a musCle tumor (e.g. myoma or myosar· dometriosis . .Metastatic bladder tumor, such as coma) or an even rarer neurog.enic tumor, suc.h as melanoma, is a possibility too. The one thing neurofibroma or neurogenic sarcoma. If this pa· against endometriosis, of course, is the ti 46 case Masson stains showed myofibrils. but we could not find cross striation. Leiomyosarcomas of bladder occur in older age patients; they are often polypoid and may be ulcerated. Occasionally, they have a pedicle; thus they are easily resectable without resort to un· necessar y cystectomy. Histologically, they often show marked edema, making the diagnosis of sar· coma difficult. The presence of hyperchromatic cells and occasional mitotic figures are lhe basis for the diagnosis of sarcoma. Dr. Mostofi's Diagnosis: LEIOMYOSARCOMA llistopathologie diagnosrs submitted: Rh.abdomyOS:lrtOmJ. . . . , ...... SO Leiomyosarcoma ...... ·~. 19 Other $areomu. , ...... 10 Othe,...... G5 Dr. Mostoli: I am sorry that I cannol agree f<\ig. l-Oblique prujettion o' r~' "lO~trnm with filling defect. with the diagnosis of rhabdomyosarcoma. I Dr. B. Weiss of Dunedin preferred squamous cell believe that it is a leiomyosarcoma. carcinoma, and Dr. Paul Berger. also or Dunedin, an adenocardnoma. Or. del Reg a to: Drs. D. Dahlin of Rochester. Minnesota, A. 0 . Severance of San Antonio and Operative Fin dings: On January 30, 1975, a Yvon LeGal of Stras bourg also made a diagnosis segmental resection of tho bladder was carried of leiomyosarcoma. Dr. Leo Low beer of Tulsa of· out; the specimen measured 8 x 9 em and contain· fered rhabdomyosarcoma; Dr. L. M. Franks of ed a tumor 6 em in diu meter. consisting of London preferred myxoid sarcom<> and hoped for necrotic and gelatinous tissue. special stains that demonstrate muscle elements. Dr. Paul A. Gikas of Ann Arbor saw only fibromatosis or low grade fi brosarcoma. Dr. Mo~toli: There is n polypoid and in· tramural tumor consisting of bands of spindle Subsequent History: From February 18 to sha ped cells separated by marked edema. The Aprill. 1975, lhe palienl received radiotherapy: a cigar·shaped nuclei contain large nucleoli total of 5400 R in 43 days to the midplane of the characteristic of bird's·eyc nuclei. The cytoplasm pelvis. On November 4, 1976, the patient was contains fuchsinophilic fibers as demonstrated by reported in good health. Cystoscopy was Mas,son. The cells lire fairly uniform. but occa· negative. sionally a hyperchromatic cell is seen. Mitoses are present (Fig. 21. The overlying mucosa is Dr. N. Rangala, Milwaukee, WI: The patient ulcerated. was seen about a week ago on routine follow·up, The histology is quite typical for leiomyosar· and she is doing well. coma. Tbis diagnosis is based on the fact that the cells are spindle, the nuclei are elongated, and Or. Whitmore: These are unusuallumors; they probably have the best prognosis of the bladder many demonstrate the typical bird's~ye nucleus of smooth muscle cell. The differential diagnosis sarcomas. They are usually solitary. and if they includes rhabdomyosarcoma in the form of sar· coma botryoides and fibrosarcoma. Vesical sar· coma botryoides is usually seen in children. The tissue is edematous. and there may be many spin· die cells as they are here. but the cell population is quite dif£erent. We sec cells resembling lym· phocytes, rhabdomyoblasts and spindle cells with c.ross striations. None of these features is present here. In fibrosarcoma the tumor is usually more ' cellular. and they usually have collagen and more mitotic figures. Masson stains are desirable to rule out fibrosarcoma and to find cross striations. In this are l~ated where a good segmental resection can operative radiation therapy would or would not be done, as was done in this case, I think the pro contribute to control is unsolved by any data of babilities of local control are excell ent. The which I am aware. tumor's propensity to metastasize (admittedly based on very limited datal seems to be relatively lte ler c ncc~ low. In other words, the principal cause of treat Das Gupta. T.D .. and CrabsiAid, H.: Melanoma of t he goniLourinary t.rac.t. J. Urol. 93:607--614. 1965. ment failure is local recurrence, rather than dis Evons. A.T .. and Bell, T.E.: Rbabdomyossrcoma of the blad· tant metastasis; a good segmental resection dor in adult patienU: report of 3 eases. J . Urol. 91:573-575. should be adequate. To what extent post- 1965. 15. MUCINOUS ADENOCARCINOMA OF THE BLADDER Contributed by Carlos Perez-Mesa, M.D., Columbia, Missouri The patient was a 76-year-old man in small with cystitis glandularis. Also, I somehow September 1956 when he complained of difficult think that this case was pegged at Dr. Mostofi and painful micturition and hematuria . There had rather than me since the radiological findings are been a loss of 10 lbs. in weight in the past year. not great, and in 1955. a year before the date of There were no abnormalities found on physical this case, Dr. Mostofi published a paper on examination. Cystoscopy revealed the presence mucous adenocarcinoma of the urinary bladder. of a friable tumor of the bladder wall. My choice is cystitis glandularis with adenocar cinoma of the bladder, but this choice has very lit Dr. Elkin: There is calcification of the vasa tle to do with the radiological findings. deferentia, so the patient must be diabetic. The bladder is small and smooth in outline, although deformed in contour. The large round filling Dr. Elkin's Impression: defect is, I hope, the water filled bag of a Foley 1) ADENOCARCINO~L\ on CYSTITIS catheter. J ust to the patient's left of the Foley GLA1'iDULARIS bag is the suggestion of a small round filling 2) TUBERCULOSIS defect; it is faint and maybe represents the tumor seen cystoscopically. There is calcification of Radiologic impressions submitted: pelvic vessels. not unusual fo r age 75 (Fig. 1). Careinoma or the bladder, ...... 33 Tuberculosis , , . , , ...... , , ...... 09 We do not have much to go on. I do not see the Sarcoma . 00 .... . 00 ...... oo • .... . 06 "friable tumor of the bladder wall " seen at Carcinoma or the pros t.aLe ...... • 10 cystoscopy. If this case were dated 1976 instead & nlgn lesions ...... , . . . L8 Others ...... oo ...... 00" •• IS of 1956, we could questio.n whether or not the pa tient bad received any type of anti-tumor Dr. Elkin: Those radiologists who said car chemotherapy in the recent past because we are cinoma of the bladder were probably deciding now seeing small bladder with the cystitis of from the cystoscopic description. Tuberculosis is cytoxan therapy, some going on to transitional a possibility, although I am not making that my cell or epidermoid carcinoma. Chronic interstitial best diagnosis. For sarcoma of the bladder, one cystitis is associated with a small bladder and would have to explain why the bladder is this ulcers but not "friable tumor of the bladder wall" small. There really is nothing on which to base and is usually found in women. Eosinophilic the diagnosis of carcinoma of t he prostate. We cystitis can be associated with a small bladder have no evidence of a bone metastasis, so I could lumen, although with a wall Again, there should not make that diagnosis. We really do not see a le be no "friable tumor:" also, eosinophilic cystitis is sion radiologically. most common in children or young adults. Dr. del Regato: Dr. Norman Rosenthal of Tam Tuberculosis typically gives a contracted blad pa also suggested a bladder tumor on cystitis der, and sometimes an. accom panying tuber glandularis. culoma in the bladder could simulate a "friable tumor" cystoscopically. This is a possible Operative Findings: On April 12, 1957. a total diagnosis. cystectomy was carried out, and an ileal loop con I wonder whether we are dealing with an duit was established with ureteral transplant. adenocarcinoma of the bladder grafted on a The surgical specimen contained a growth cystitis glandularis. The bladder lumen can be 3.5 x 1.5 em in diameter, apparently superficial, 48 demonstration of invasion, but we have felt that lakes of mucin should arouse·· suspicion of malignancy . These t umors have about the same type of behavior as corresponding transitional cell carcinoma except that they are not radiosen· sitive. The third alternative, secondary adenocar cinoma of the bladder, is more uncommon. The pa· tients usually have had the clinical manifestation of an extra vesical primary tumor. Most common primary sites are the neighoring organs. There is still another adenocarcinoma of blad der , mesonephric adenocarcinoma, which resembles clear cell carcinoma of the kidney or li'ig. I- Smail derormed bladder with. rounded defect. the ovary. These are extremely rare. Let me also add that in a number of transitional cell car which on cross section was rubbery gray-white cinomas, we do see glandular or tubular areas, and presented tiny cysts. but we categorize these as transitional cell car cinomas with glandular or tubular areas. Dr. Mostofi: The section shows a bladder with Dr. Mostofi's Diagnosis: a mucinous adenocarcinoma which is papillary and superficially infiltrating (Figs. 2, 31. 'rhe adja MUCINOUS ADENOCARCINOMA cent mucosa shows proliferative and metaplastic Histopathologic diagnoses submitted: changes with Brunn's nests, cystitis cystica, glan· Adenotareinonta . . , . , ...... , . . , . 48 dularis and mucous metaplasia. In deeper portion, Mucinous adenocarcinoma ...... 1"7 several lakes of mucin are seen (Fig. 4). There is Metastatic adenocarc-inoma . .. . , ...... 15 marked lymphoblasmatocytic infiltration of Urachal earcinoma ...... 05 lam ina propria. Cystitis glandularis .. . ~ ...... 05 Others ...... Od The differential diagnosis here is between a urachal adenocarcinoma, a primary vesical Dr. Mostofi: The majority made the diagnosis adenocarcinoma, and a metastatic adenocar of adenocarcinoma; we are all in agreement with that. As far as the diagnosis of metastatic cinoma of bladder. adenocarcinoma or urachal carcinoma, I will come Urachal adenocarcinomas occur in the dome or back to those later. There is more t han cystitis the anterior wall, are principally intramural, may glandularis here; as you know, the cells are ulcerate and extend into the bladder lumen: they neoplastic, ·and there is definitely a tumor. The extend along the ramifications of urachal tract most common finding of adenocarcinoma of the through the space of Retzius to the umbilicus. In bladder is in relationship with transitional cell the present slide the tumor is polypoid and confin carcinomas. About eight to ten percent of transi ed to the mucosa and superficial muscle. It is not tional cell carcinomas will show glandular areas intramural. We have no information to indicate in deeper portions. If the tumor is in that there was a tumor in the dome or anterior the dome or anterior wall of the bladder, we wall of the bladder, or that the anterior ab· must think oJ urachal adenocarcinoma; the domina) wall was involved, so we can rule out an characteristic feature of urachal adenocarcinoma urachal adenocarcinoma. Let me say that urachal is that the tumor is predominantly intramural adenocarcinomas are late in being detected and with extension into the bladder and ext.ension all are often incompletely removed, so there is recur rence. In such cases, not only t he involved area of F'ig. 2- Biadder with adenoc