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CALIFORNIA TUMOR TISSUE REGISTRY

California Tumpr Tissue Registry c/o: Department of Pathology and Human Anatomy Lorna Linda University School ofMedicine 11021 Campus Avenue, All 335 Lorna Linda, California 92350 (909)" 824-4788 FAX: (909) 478-4188 Target audience: Practicing pathologists and pathology residents.

Goal: To acquaint the participant with the histologic features of a variety of benign and malignant and tumor-like cOnditions. . Ob!ectlve: The participant will be able to recognize morphologic features of a variety ofbenig!l and malignant neoplasms and tumor-like conditions and relate those processes to pertinent references in the medical literature.

Educational methods and media: . Review of representative glass slides with associated histories. Feedback on consensus diagnoses from participating pathologists. Listing of selected references from the medical literature.

Principal faculty: Weldon K. Bullock, MD Donald R. Chase, MD

CMECredlt: The CTTR designates this activity for up to 2 hours of continuing medical education. Participants must return their diagnoses to the CTTR as documentation of participation in this activity.

Accreditation: The California Tumor Tissue Registry is accredited by the California Medical A~sociation as a provider of continuing medical education. 'l .

CONTRIBUTOR: Lorna Linda Pathology Group (cz) CASE NO. 1 - MAY 1997 Lorna Linda, CA

TISSUE FROM: Left neck ACCESSION #28031

CLINICAL ABSTRACT: This 80-year-old female presented with a left-sided neck mass which did not appear to enlarge ·over the next six months. A left radical neck dissection was performed.

GROSS PATHOLOGY: The 240 gram, 12.0 x 12.0 x 5.0 em left neck contents specimen was largely replaced by tumor. A 10.0 x 6.0 x 2.5 em portion of firm tan tumor extended from the midline into the lateral neck, apparently replacing the thyroid which was riot identifiable. A 4.0 x 4.0 x 3.0 em area of tumor was wrapped around the trachea and portions of the larynx.

CONTRIBUTOR: Robert E. Riechrnann, Jr., M.D. CASE NO. 2 - MAY 1997 Covina, CA

TISSUE FROM: Right mandible ACCESSION #28036

CLINJC.i\L ABSTRACT: This 29-year-old male presented with an expansile mass of the right mandible. Radiographic and CAT scan examination revealed a 4.5 x 4.0 em mass involving the entire right ramus and partial body of the mandible.

GROSS PATHOLOGY: A 10 x 4 x 3 em curved, crescent-shaped piece·of hard white was expanded in the ·midportion by a 7 x 4 x 3 em mass composed of homogeneous dense white tissue within which there was an irregularly-shaped area of central cyst formation. • CONTRIBUTOR: Chisa Aoyama, M.D. CASE NO. 3 - MAY 1997 Sylmar, CA

TISSUE FROM: Pelvic mass ACCESSION #28210

CLTNICAL ABSTRACT: This 21-year"'Old female presented with a 4 month history of intermittent episodes of · diaphoresis, headaches, blurred vision, shortness of breath, palpitations and fatigue. Her blood.pressure during physical examination was elevated (as high as 203/116) and labile. Twenty-four hour urine catacholamines showed normal levels of epinephrine with markedly elevated levels of norepinephrine, VMA and dopamine. cr scan of the abdomen and pelvis showed a large mass in the pelvis behind the uterus. The mass was resected along with a portion of adherent sigmoid colon. There were multiple nodules in the omentum and mesentery.

GROSS PATHOLOGY: A 11.5 x 8.5 x 7.5 em multinodular yellow-tan mass was adherent to the serosal surface of a resected segment of bowel.

SPECIAL STAINS: Chromogranin: diffusely positive

CONTRIBUTOR: Mark Janssen, M.D. CASE NO. 4 - MAY 1997 Anaheim, CA

TISSUE FROM: Left groin mass ACCESSION #28018

CLTNICAL ABSTRACT: This35-year-old Asian-American male presented with a 2-3 year history of a mass in the left groin area. Physical examination showed a 3.0 x 1.0 em subcutaneous hard lump in the groin. This was excised.

GROSS PATHOLOGY: A 5.2 x 2.5 cr:n ellipse of skin, and cut to a depth of 2.0 em, had a central, slightly raised area 3.5 x 2.4 em in diameter.

SPECIAL STAINS: CD34: negative Actin: negative S-100 protein: positive CONTRIBUTOR: David Huebner, M.D. CASE NO. S • APRIL 1997 Fontana, CA

TISSUE FROM: Abdominal wall ACCESSION #28032

CLINICAL ABSTRACT: Ten years ago this 76-year-old female had a total abdominal hysterectomy and bilateral salpingo­ ·oophorectomy for stage 1, grade II endometrial carcinoma, followed postoperatively with adjuvant radiation. Two years ago she presented with a 3 x 4 em mass in the abdominal wall. This was resected and she was lost to follow-up. She.now presents with an enlarging 6.0 x 9.0 x 15.0 em abdominal wall mass. The mass was again resected.

GROSS PATHOLOGY: The specimen consisted of a.28.0 x 7.0 em ellipse of skin, with underlying fibroadiP.Ose tissue and . Within the muscle was a 23.0 x 11.0 x 7.6 em relatively well-circumscribed, lobulated, rubbery, firm pink-tan mass.

SPECIAL STAINS: Keratin cocktail, low molecular weight keratin (CAM 5.2), B-72.3: positive EMA, Vimentin: focal positivity Leu-M1: focal luminal positivity (tumor cells essentially negative) CDA, AFP, SlOO, Chromogranin: negative

CONTRIBUTOR: Chisa Aoyama, M.D. CASE NO. 6 - MAY 1997 Sylmar, CA

TISSUE FROM: Abdomen ACCESSION #28026

CLINICAL ABSTRACT: This 39-year-old Caucasian female had childhood polycystic kidney disease with recurrent nephrolithiasis and chronic pyelonephritis leading to a right nephrectomy at age 18. Atthat time she had a right oophorectomy and hysterectomy for ovarian carcinoma. The tumor pathology is not known but there is a possible history that the tumor secreted androgens. she now presents with left flank pain. Work-up found a large abdominal mass which was debulked.

GROSS PATHOLOGY: The 2.3 kg, 20.0 x 20.0 x 10.0 em specimen consisted of multiple fragmented pieces of white­ tan tissue with focally hemorrhagic and myxomatous change.

SPECIAL STAINS: Vimentin: positive EMA: positive Desmin: negative CONTRIBUfOR: Charles C. Osborn, M.D. CASE NO. 7 - MAY 1997 Glendale, CA

TISSUE FROM: Uterus ACCESSION #27962

CLINICAL ABSTRACT: This 50-year-old female was found to have an enlarged uterus and a hysterectomy was ·performed.

GROSS PATHOLOGY: Within the uterus was an 11.0 em diameter myometrial mass. It was very edematous, hemorrhagic and friable and appeared to be well circumscribed.

SPECIAL STAINS: CD-34: negative Desmin: positive

CONTRIBUTOR: Dhnrnm M. Ramnani, M.D. CASE NO. 8 - MAY 1997 Dallas, TX

TISSUE FROM: Spleen ACCESSION #28193

CLINICAL ABSTRACT: This 13-year-old Caucasian male has had a progressively worsening splenomegaly, which had been symptomatic, causing some respiratory embarrassment and anorexia. ACT scan of the abdomen revealed compression of the pancreas and stomach. A subtotal splenectomy was performed.

GROSS PATHOLOGY: The 1533 grams portion of spleen was 22.0 x 9.5 x 7.5 em. The specimen was intact except for an 11.0 x 10.0 em area close to the hilum, and the inferior pole where parenchyma was exposed. The specimen was serially sectioned in 1.0 em thick slides revealing firm, pale, red-brown parenchyma. No focal lesions were identified.

SPECIAL STUDY: Electron microscopy showed the splenic macrophages to be distended by well-defined cytoplasmic lysosomes containing small irregular whorls of lamellar material consistent with sphingomyelin. CONTRIBUTOR: Thomos Heinz, M.D. CASE NO. 9 - MAY 1997 Oronge, CA

TISSUE FROM: Liver ACCESSION #28105

CLINICAL ABSTRACf: This 9-month-old Caucasian female presented with vomiting, mainly at night. Physical .examination revealed a large abdominal mass. Further workup showed an elevated alpha fetoprotein of around 30,000. Ultrasound, chest cr, MRI, and hepatic angiography demonstrated a very large right hepatic tumor which displaced what appeared to be the right portal vein into the left lobe of the liver. An extended right hepatic lobectomy was performed.

GROSS PATHOLOGY: The specimen consisted of a 12.5 x 10.0 x 8.0 em, 427 gram portion of liver with variegated pale tan to red-tan glistening capsule. Within the liver was a 9.5 x 85 x 7.5 em, well-circumscribed variegated pale tan to gray-tan mass.

CONTRIBUTOR: F. Azlzl, M.D. CASE NO. 10 - MAY 1997 Fontana, CA

TISSUE FROM: Right thigh ACCESSION #28085

CLINICAL ABSTRACf: This 21-year old female presented with a right thigh mass measuring about 20 em, which had been growing slowly for approximately two years.

GROSS PATHOLOGY: Four pieces of soft gray-pink, friable tissue, varied from 2.0 to 2.5 em in greatest diameter.

SPECIAL STAINS: Cytokeratin: positive EMA: positive S-100: negative CAUFORNl<\ TUMOR TISSUE REGISTRY

SUGGESTED READING (General Topics from Recent Literature): Relationship BetwCCJJ Pelvic Jym1Jh Node I.nvolvement and Other Disease Sites in Patients with Ovarian Cancer. Gynecol Ouco/1997; 65: 164-168. Corrclntlon of Body Fat Distribution with Grade of Endometrial Cancer. Douchl T, ljuin B, Nal

INLAND CR!\IERS!DEISAN BERNARDINO> • Mc~J~Static adenocarcinoma, NOS (?medullary carcinoma of thyroid) (I); Medullary thyroid carcinoma (I); Hunhle cell can:inoma (I); Papillary carcinoma or th)'lroid with a solid component (I). Orlcoe)'tic carcinoma (1), Undifferentiated carcinoma, thyroid (1).

Giantcarcinoma c:cli carcinoma (1); Poorly or differentiated thyroid. carcinoma (1). ~~~~~~~~[~-• Poorly differentiated (insular) carcinoma, thyroid (6). ~ adenocarcinoma (5). ~~~~~~~~~~~~ • Undifferentiated (anaplastic, sarcomatoid) careinoma ofthyroid . ~ - Anaplastic CA, NOS (7); Anaplastic CA, arising from insular CA (4); Hurthle cell CA (1). ~~;A;~&!JAE~(Ql~~~l!illill · Anaplastic carcinoma, thyroid. ~:§:lf~~~~:;~~~= · Poorly differentiated caicinoma ..,~~~tife:::Ja;~ carcinoma, r/o thyroid medullary carcinoma (2); Malignant nec1p1asm, thyroid. medullaty carcinoma vs paraganglioma, malignant (1). BAX AREA • Medullary carcinoma ofthyroid (anaplastic carcinoma developing from a follicular carcinoma cannot be excluded) (3). SACRAMENTO - Poorly differentiated thyroid carcinoma with Hurtble c:cli and anaplastic features. ARIWNA ITucsonl • Poorly differentiated thyroid carcinoma [Insular carcinoma). WYOMING • Anaplastic thyroid carcinoma TEXAS ffil Paso) - Anaplastic carcinoma ofthyroid (2). NEW MEXICO CAibuquerouel - Medullazy carcinoma with anaplastic features. NEBRASKA

DIAGNOSIS: POORLY DIFFERENTIATED TIIYROID CARCINOMA WITH HURTBLE CELL FEATURES 1'96000, M82903

NOTE: Inununostains for keratin and thyroglobulin were positive. Stains for chromogtanin, synaptophysln and S-100 protein \vere negative. Electron microscopy showed microacinus formation.

REEER£NCES: Watson RG, Brennan MD, Goellner JR. et al Invasive Hurthle Cell Can:inoma of the Th)roid Natural History and Mllllllgemenl Mayc Cli11 Proc 1984; 59: 851-855. Gan:angiu ML, Bionchl S, Savino D, et at Follicular Hurthle Cell Neoplasms of the Th)'TOid Gland. A Study or IS3 C.S.. Cmct~r 1991;68: 1994-1953. Mueller-Oaertner HW, Bnac HT, Rebpemning W. Prognootic Indices for TIJmor Rclapse andTumor Morlality in Follicul.vThyroid Careinoma. Cancer 1991; 67; 1903-1911 . CASE NO. 2, ACCESSION NO. 28036 MAY 1997

INLAND - Ossifying (3); Fibrous dysplasia (I), Desmoplastic fibroma (2). CAMARILLO Wviso Group) - Non-ossifying fibroma. \IENTIJRA CUnilabl - Fibrous dysplasia (2). PLEASANTON/FREMONT - Desmoplastic fibroma vs fibrosarooma (6). LONG BEACH - Fibrsseous lesion consistent with ossifying fibroma (5). QXNARD CSt. Johns Recional Medical Ctrl - Fibrous dysplasia/rio ossifYing fibroma. SAN DIEGO SSeous lesion, most consistent with fibrous dysplasia (14). SANTA BARBARA CCouage Hosnita!l - Ossll)1ng fibroma. SANTA CLARA - Fibro-osseous lesion. ARlZONA (f)Jcson l - Desmoplastic fibroma. WYOMING - Low grade of bone. TEXAS lEI Paso) - Fibrous dysplasia (2). NEW MEXICO CA!buoueroue) - Desmoid tumor. NEBRASKA CCreigluonl - Ossifying fibroma. FLORIDA CBcthesda Naval Med Ctrl - Fibrous dysplasia. MARYLAND CBethesda Naval Medical Center) - Ossifying fibroma (14). NEW HAMPSHIRE (Manchester) - Odontogenic fibroma vs fibrous dysplasia (I); Fibrous dysplasia (2). NEW )ERSEY

DIAGNOSIS: , RIGHT MANDIBLE TI0180, M76100

.• CONSULTATION: Roben B. Brannon, MD., ChaiiiiUUl, Department ofOral Patl10log;y, AFIP, Washington, D. C. "Fibromatosis."

REFERENCES: Fu YS, Perz.in and Penin KH. NonepitheliBI Tumor of the Nasnl Cavity, Para-Nasal Sinuses and Nasopharynx. A Clinicopathologic Study VI. Fibrous Tissue Tumors. Fil::rorna, Fibromatosis, Fi:bro5arcoma. Ccnctr 1976; 37: 2912 Takagi M, Yamamoto H, Mega H. etal. Hetetos=ity in the Gingival Fil::romatosis Canur 1991; 68: 2202-2212. Zain RB and Fct YJ. Fibrous Lesions of!he Gingiva. A Histopathologic Analysis of204 Cases. Oral Surg Oral Med, Oral Patltol 1990; 70(4): 466-470. Oshiro U, Fukuda T, Tsuneyoshi M Fibrosarcoma Versus Fibromatosis and Cellular . A Coolparativc Study of their Proliferative Activity Using Proliferating Cell Nuelcar Antigen, DNA Flow Cyt

INLAND • Melastatic pheochromocytoma (3); E.xtrn adrenal paraganglioma (1); Paraganglioma (2). CAMARILLO (Alviso Grouol • Organ ofZuekerkandl catcinoma. VENTURA Nnilabl - Pheochromocytoma (2), PLEASANTQNIFREMONT • Paraganglioma (6). LONG BEACH • Pheochromocytoma (5 ). QXNARD CSt Johns Regjonal Medjcal Ctrl • Pheochromocytoma (extra aclrcnal). SAN DIEGO - Pheochromocytoma (2). NEW MEXICO CAibu0uernuel - Carcinoid tumor. NEBRASKA (Creighton University) • Pheochromocytoma. FLORIDA

DIAGNOSIS: 1\-lALlGNANT PARAGANGUOMA, PEL VIS T¥6000, M86801

Note: Not all study set slides showed vascular involvement or vaswlar invasion.

FOLLOW-UP: A month later the patient expired. No autopsy was perfonncd.

REFER£NCES: TIUnors of the Extro-Adrenllll'araganglion System (Including Chemoreceptors). AtlaJ o/Tumor Pathol, S..:Ond Series, Fascicle 9, AFTP. Washington, DC. 38-194. Linnoi.la RJ; Keiser HR, Steinberg SM and Lack EE. Histopathology of Benign Versus Malignon! Sympathetic Adrmal P0t11gangliomas. A Clinieopatholo&ic Study of 120 Cases, Including Unusual Histologic Features. Hum Patloo/21: 11 68-11 &0. CASE NO. 4, ACCESSION NO. 28018' MAY1997

lNLAND

DIAGNOSIS: GRANULAR CELL TUMOR, LEFT GROIN TY7000, M95800

REFERENCES: Le Boil PE. Barr RJ, Burall S, Metcalf JS, et al. Primitive Polyposis Gnmular-€ell Twnor and Other Cutaneous Granular Cell Neoplasms o(ApparentNonnewal Origin. AmJSuf'8 Potho/1991; 15(1): 4~58. Lee J, Bhawan J, W~x F and Farber J. P.lexifonn Granular Cell Twnor. A report ·ofTwo Cases. Am J Dtmriztopadu>l 1994; 16(5): 537-54 I. Bakos L. Multiple Cutaneous Gnmular Cell Tumors with Systemic Defects. A Distinct Entity? lnt J D.!m~aUJ/1993; 32(6): 432-435. Patnaik AK. Histologic and Immunohistochemical Studies ofGranular Cell Tumors in Seven Dogs, Three Cats, One Horse, and One Bird Vet Parho/1 993; 30(2): 176-185. Kurtin PJ and Bonin DM Immunohistochemical Demonstmtion of the Lysosome-Associated Glycoprotein CD68 (KP·I) in Granular Cell Iumors·and Sehwannomas (See Conunents). Hum Patho/1994; 25(11): 1172·1178. Sayer HP, Metze D, and Keel H. 'Granular Cell Dcnnatofibroma. AmJSurg Patholl991; 19(2): 168-173. CASE NO. S, ACCESSION NO. 28032 MAY 1997

INLAND CRMRSIDEISAN BERNARDINO> - MetaStatic adenocarcinoma clw endomelrial origin (2); (malignant mixed mullerian tumor) (I); Mewaatic: c:arcinoma (1); Poorly diffcrentiat.ed adenoe31cinoma perhaps from previous malignancy (clear cell endometrial) (2). CAMARILLO (Alviso Group) - Adenoac:anlhoma. VENTIJRA - Metastatic adenocarcinoma (2). NEW MEXICO CAlbuqueroue) - Poorly differentiated carcinoma, probably adenocarcinoma. NEBRASKA CCrej@ton University) - Adenocarcinoma (metastatic vs impla.nted). FLORIDA

.DIAGNOSIS: POORLY DIFFERENTIA TED ADENOCARCINOMA WITH CLEAR CELL FEATURES, PlWBABLY OFMUL:LERIAN ORIGIN, ABDOMlNAL WALL Il(4300,M83103.

FOLLOW-UP: The patient had a recurrence six months post-operatively, whicb was nxxcised.

REFERENCES; None. CASE NO. 6, ACCESSION NO. 28026 MAY 19'>7

INLAND CRIVERSIDEISAN BERNARDINO> - Ncplvoblastoma (2); Malignant neuroectodermal tumor (I); Adult Wilms tumor (I); Endodennal sinus tumor (I); Undifferentiated crucinoma (I). CAMARILLO < cord stromal twnor (5); Granulosa celltwnor (3). SANTA CLARA - Juvenile granulosa cell tumor. SANTA ROSA - Poorly differentiated Sertoli-Leydig celltwnor (I); Gyandroblastoma, residual, recurrent by blstory (I); Sertoli Leydig cell (!). • BAY AREA - Malignant sex cord stromal tumor (2); Carcinosarcoma (I). SACRAMENTO CUC Davis> - lmmatll!C teratoma. AR!ZONA Cfucsonl - Low grade malignant teratoma. WYOMING - Poorly differentiated Sertoli-Leydig cell tumor- metaslatic. TEXAS CEI I'll so) - SertoU-Leydig oell tumor, poorly differentiated (2). NEW MEXICO (Aibuauerauel - Sertoli-Leydig cell tumor intermediate differentiation. NEBRASKA - ? Sertoli cell vs immatll!C teratoma. CONNECDCVT - Sertoli-Leydig cell tumor of intermediate grade. AU§TRAL!A

DIAGNOSIS: SERTOLl-LEYDIG TUMOR, ABDOMEN TY4100, M86JIO

CONSULTATioN: Dr. Fu from UCLA foels that the differential diagnosis should include "Immature Teratoma and Sertoli-Leydig Cell Tumor."

REfERENCES: Young RH and Seully RE. Ovarian Sertoli-Leydig Cell Tumon. A Clinic:opelhological Analysis of207 Cases. Am J Surg Patlroll985; 9(8): S4l-569. CASE NO. 7, ACCESSION NO. 27962 MAY1997

INLAND CRIVEBSrDEISAN BERNARDINO> - Endomclrial stromal nodu«: (I); Stromal nodule; Cellular (I); Vascular leiomyoma (I); Epithelioid leiomyoma, plexiform type (I); Angiomatosis (I). CAMARILLO (Alviso Grouol - Angiosarcoma VE!:{ruRA CUnilabl - Stromal nodule (1); Endometrial stromal nodule vs low grade stromal sarcoma (1). PLEASANTON/FREMONT - Leiomyoma, cellular and plexifonn (4); Stromal nodule (2). LONG BEACH - Lymphangio-lelomyoma (3); Pericytoma (2). OXNARD - Endometrial stromal nodule. ARIZONA (Aibugucmue) - Vascular leiomyoma. wyOMING - Low grade endometrial slromal sarcoma. TEXAS CEI Paso) -Endometrial stromal sarcoma, low grade (2). NEW MEXICO - Endometrial slromal sarcoma, low grade. NEBRASKA

DrAGNOSIS: ENDOMETRIAL STROMAL NEOPLASM, PROBABLY STROMAL NODULE T8400Q, M8000l

CONSULTA TIQN: Richard Kempson, MD., Stanford University Health Center: Endometrial stromal neoplasm, probably stromal nodule (based mostly upon its circumsaiption).

FOLLOW-UP: No recurrence of the tumor after one year.

REFERENCE$: Chang KL, Crabtree GS, Lim-Tan SK, d aL Primary Uterine Endometrial Stromal Neoplasms. A Clinicopathologic Studyofli7Cascs. AmJSurgPathoJ 1990; 14(5): 4JS-438. Kempson RL and Hendrickson MR. Pure Mesenchymal Neoplllsm of the Uterine Co!pus. Selected Problems. Semin Diag PatltoJ 1988; 5(2): 172-~98. CASE NO. 8, ACCESSION NO. 28193 MAY1997

INLAND CR! VERSIDEISAN BERNARDINO> • Niemann-Pick disease {5); Lipid storage disease (Histology not characteristic of citl1er Gaucltcr or Niemann Pick). CAMARILLO CAiviso Grouol • Histiocytosis X. VENTURA aJnilabl - NiemaM Pick (I); Lipid histiocytosis c/w Niemann Pick disease (I). PLEASANTQNIFREMONT • Sphingomyelin Lipidosis (Niemann-Pick disease) (6). LONG BEACH - Sphingomyelin lipid storage disease (5). OXNARD CSt Johns Reruonal Medical Ctrl - Lipid storage histiocytosis c/w Niemann Pick disease. SAN DIEGO CNaval Me4 Ctrl • Niemann-Pick disease, type B (15). SANTA BARBARA (Cottage Hospital) • Niemarut Pick disease. SANTA CLARA (Lorna Prieta Group) • Niemann-Pick disease, type B. SANTA ROSA • Niemarut-Pick disease (2); Sphingomyelin-lipidosis (Niemarut-Pick) (I) BAY AREA - Ncimann·Pick disease (3); (?Fabry's). • SACRAMENTO aJC Davis> - Niemann Pick disease, type B. ARJmNA Cfucsonl • Nicmarut·Pick disease, benign variety. WYOMING • Niemarut Pick disease. TEXAS CEI Pasol • Niemarut Pick disease (2). NEW MEXICO CAibuguerauel - Niemann Pick disease. NEBRASKA • Sphingolipidosis (Niemann Pick disease (14). NEW HAMPSHIRE CMancheslerl - Niemarut Pick disease. NEW JERSEY COyerlook Hosoj!a! Swnmitl - NicmaM Pick disease (3). NEW YORK • Niemaru1-Pick disease (4). MASSAC!fUSETIS (Berkshire Med Ctrl - Niemann Pick disease, type B. MAINE CBangorl • Niemann Pick disease. CONNECTIC\IT • Gaucher's disease. AUSTRALIA CSydnevl • Niemann-pick disease. JAPAN CShimada·Kvotol - Niemann pick disease.

DIAGNOSIS: NIEMANN-PICK DISEASE, SPLEEN T07000, DI420

FOLLOW-UP: A year an.d a half after surgery the patient is asymptomatic and doing weU.

REFERENCES: Dawson PJ Md Dawson CT. Adult Niemann-Picl: Disease with Sea-Blue Histioeytes in the Spleen. Hum Patho/1982; 13: 1115-1 \20. SilversteinMN, Ellefson RD,and Ahem ET. The Syndrome of the Sea-blue Histiocyte. N EngJMcd 1970; 282: 1-4. Brunning RD. Morphologic Allerations in Nucleated Blood and Marrow Cells in Genetic Disorda's. Hum Patho/!910; 1: 99-124. Brady RO. Sphinomyelin Lipidooes. Niemann-Picl: Disease. In Stanbury JB, Wyngaarden JB, Fric:kricl:soo DS, Goldstein JL, Brown MS {Ed$). Tite Me!Abolicllasis of Inherited Disease. New York 1983, McGraw-Hill Book Company: 831-841. CASE NO. 9, ACCESSION NO. 28105 MAY 1997

INLAND !RIVERSIDE/SAN BERNARDINO> - , favorable fetal pattern (I); Hepatoblastoma (4), Adenoma (I). . CAMARILLO - Hepatoblastoma, epithelial type (14). NEW HAMPSHJRIHManchesterl - Hepatocellular carcinoma (3). NEW JERSEY

DIAGNOSIS: HEPATOBLASTOMA T5600.0, 1\<189703

REFERENCES: Conran RM, Hitchcock CL, Maclawi W, et 81. Hepatoblastonia. The Prognostic Significance ofHistologic Type. Pediacarpatho/1992; 12: 167-183. Go112ales-Crussi F, Upton MP and Mauer HS. Hepotoblastoma. Attempt at Characterization of Histologic Subtypes. Am J Surg P~tho/1982; 599.§12. Lacke E, Ncave C and Vawter GF. Hepotoblastoma. A Clinical and Pathologic Sltldy of54 Cases. Am J Surg Pathol 1982; 6: 693-705. Saxena R. Leake, JL, Shafford EA, Thwenport M. et al. Chemotllempy Effects on Hepotoblastoma. AmJSurg Pathol .1993; 17(12): 1266-1271. CASE NO. 10, ACCESSION NO. 28085 MAY 1997

!NLANQ • Monophasic, synovial sarcoma, fibrous type (2); Synovial sarcoma (I); Epithelioid sarcoma (I); Epithelial sarcoma-fibrosarcoma pattern (I); Malignant schwannoma (I). CAMARILLO

DIAGNOSIS: MONOPHASIC SYNOVIAL SARCOMA, RIGHT TIDGH T¥9100, M90403

CONSULTATION: Christopher D. M Fletcher, M.D. Harvard Mcdieal School. "Monophasic Synovial Sarcoma."

REfERENCES: Oda Y, Hashamoto H. Takeshita Sand TS>mcyoshl M. The Prognostic Value oflnununOOi.stocl!emical Slaining for Pro1ifemling Cell Nuclear Antigen (PCNA) in Synovial San:oma. Ct:ncu 1993; 72: 47&-4&5. Lopes 1M, Bjeri;ellagen B, Holm R, ctal. Immunohistochemical Profile of Synovial Sarcoma with Emphasis on the Epithelial-Type Differentiation. A Study of49 Primal)~ Tumcn, Recurrences and Metastases. Parhol Ru Pracr 1994; 190: 168-177. MacKenzie DH. Monophasic Synovial Sllrcoma. A Histological Entity? HIJtoparho/1977; 1: 1S1-157. Miel!ulcn M. Keratin Subsets in Spindle Cell . Keratins are Widespread but Synovial Sarcoma Contains Distinctive KC!lltin Polypeptide Pauern mld Desmoplakins. Am J Parho/1991; 138: 505-S13. Qda Y, Hashamoto H, TSUileyoshi M ruld Takcshila S. SUIVival in Synovial Sarcoma. A Multi variant Study of Prognostic Factors mth Special Emphasis on Comparison Bctwocn Early Death and Lo03-Tenn Sum val. Am J Surg Potho/1993; 17: 3544.