PATHOLOGISTS' CLUB OFNEWYOBK

Pkf.SID.tlNT FkEI'J B. SMJlH. M.D. Dm'AJ:l'lroQ_lST OF PAniOLOGY ST. VIN(."0n"S ltoSPrrAL MEETING W 9r.'BT llnl I-1'P.Err ~"EW YOJU(. NY tcOII

VIC£ PU!fll)0,.,. JQ.\.~ 0. ..I()HRJ, M.D. A.VA'TONlC PAntCU.oGY Bssn!lN·WI:!Il.l!Jt U06PrrAL WJ EA.'ITCIIllS11!X ROAD DATE: Thursday, October 10, 1996 BR~NY 10ol61

SECitP.TARV•TfUY\SUil.ER S'JYL1AN05 J..OMVAROM, M.D. PLA CE: St. Luke's - Roosevelt Hospital Center DEJ'All'l'JomNTOP PAnlOLOGV n:. ~ JIOSPrt..U. l 000 lOth Avenue Cf v.uT svm snw- ~"£W YOilK. NY 10010 New York, New York 10019

HOS T: Neville Colman. M.D.. Ph.D. Director of and Laboratory

INFORMATION: Harold Gaetz, MD 212-523-8625

RECEPTION & DINNER: Cafeteria, l st. Floor (New Building) 5:15PM-6:45PM

S CIENTIFIC SESSION: Conference Room B 2nd Floor (New Building) 7:00PM -9:00PM

Directions:

By Subway: Take any subway to Co lumbus circle, walk west 2 blocks along 58 th Street to 1Oth Avenue.

By Car: Park on 58th Street between 9th and I Oth Avenues; 59th Street, South Side; 60th Street Ncar 9th Avenue. Parking: Hospital Parking lot, 59th Street between 1Oth and II th Avenues. North Side, down form corner CASE HISTORIES- PATHOLOGISTS' CLUB MEETING, October 10,1996 (1\'ot necessarily discussed in this order)

CASE#l A 69 year old female presented with abdominal pain, nausea, vomiting diarrhea 9651734 and a recent 30 I b weight loss. There was a palpable liver 2cm below the costal margin. Extensive periaortic caval adenopathy and several low attenuating lesions in the liver were noticed. The latter was biopsied.

luvited Speaker: Dr. Frizzera Host Speaker: Dr. Abebe

CASE#~ A 29 year old afebrile male presents with a persistent cervical mass. Past 96ROI710 m ed~ c~d h.ii:to~ r; and ];e:vi.,..w of symptoms llre non contributory.- TheJnass f'liled to involute following antibiotic regimen. It was excised.

fmiited Speaker: Dr. Strauchen Host Spet1ker: Dr. Ames

CAS£#3 A 35 year old male known HIV(+) presented with SOB, fever and thick-walled '54104-88 non air fluid cavity RUL. Segmentectomy performed.

Invited Speaker: JolmProtic, MD Host Speaker: H. Gaetz, MD

CAS£#4 A 54 year old female with a right pelvic mass at abdominal exploration was found S-10166-94 to have an J.8cm X 14cm inta.ct encapsulated mass with attached normal oviduct. Cut surface exhibited a tnixed light tan solid and smooth-walled serocystic (0.5- 2cm) composition. A section submitted. The uterus, left adnexa, omentum and iiiac !ymp:t nudes \VCrc t"n:e uftumo1's and significanq)aihologic changes. • Invited Speaker: Thomas Wright, MD Host Speaker: H. Gaetz, MD

CAS£#5 A 42 year old 1:-UV(+) male presented with a tense malignant ascites and 96R04650 peritoneal nodules on CAT scan. There was no periaortic adenopathy, hepatic or pulmbnary masses. An omental biopsy was performed.

I11vited Speaker: Dr. Feiner l11vited Host: Dr. Ames l PATHOLOGISTS' CLUB OFNEWYORK

.MINUTES OF MEETfNG PR.ESID~I F'RJ:1) B. SMI11L ~D. OF.7ART'M:I;NTO? f'ATHOOOOY ST. vtNCENT'S HOSPITAL ROOSEVELT HOSPITAL t$) \J..EST IITII STREET N"f:\A' YORK. !

. 'vlC! ;R.E:siD.eoT JOAN G. J~"ES. M.D. A."i,\TOMIC PA'lllOLOGV Eih'S.1l!ll

SECRETARY·'lllE'AStiREJt our host, Dr. Coleman for his hospitality. Dr. Gaetz of Sm.IA.~O& lOMVM'tl)llJS, M.D, Roosevelt Hospital chaired the Scientific Session. Members DEP.6,R.TfdENT O'P PATHOLOG-Y 51. CL\Il£'S HO!;fn'AL unanimously approved the membership application of Drs. ~ W",:STl21\"D STR.EET' r,&W YORX, NY 1(0\9 Helen Feiner, Kasturi Das and Carol Fehmian.

Case I (96-S-1734) Invited discussant: Dr. Glauco Frizzera, NYU Medical Center Host discussant: Dr. Liu Dr. Frizzera described the morphology of the liver nodules in this 69 year old woman, highlighting the variety of forms, including large cells with bizarre multinucleation, the presence of sinusoidal infiltration, and extramedullary hemopoiesis outside the nodules. He emphasized that the histologic appearance was insufficient tor diagnosis. While he could suggest leuke~olia, the differential diagnosis would have to be based on immunohistologic evidence. Among hemopoietic cell markers, CD 43 was demonstrated in the nodules, sinusoidal and multinucleated cells. This membrane protein, a leukosialin. occurs in most hemopoietic cells .and is also detectable by MTI, Leu 22 and L60 stains Small vessel endothelial cells and vascular tumors do not exhibit CD43. Obviously megakaryocytic cells in this tumor also showed CD43. Stains for T and B markers were negative. Tumor ceHs also showecj the bone marrow progenitor marker CD34 and Factor VIII. CD34 is found in 40% of cases of AML, 70% ofB-ALL and few ofT-ALL. Of non-hemopoietic cell markers, vimentin appeared in a few ceUs, but no cytokeratin or S-1 00. Therefore, Dr. Frizzera concluded, his diagnosis was extramedullary myeloid leukemia. bett~r known as granulocytic sarcoma or chloroma. This may exist in several fonns:.Primary EML-with no prior sign of disease in marrow, skin. lymph nodes or spine; most culminRte in AML or acute monocytic leukemia . . EML at the diagnosis of ANLL, or as an isolated condition. If left untreated, primary EML will evolve into AML, but with only one third will do so. Diagnosis may best be confinned with the demonstration ofCD43 (in 97%), myeloperoxidase, lysozyme 81 , CD68 and the Leder stain.

Dr. Liu offered the additional information that periaortic lymph nodes were enlarged. Diagnoses under consideration were carcinoma, lymphoma, malignant histiocytosis. and sarcoma. The finding of CD41 and CD61 pointed to megakaryoblastic leukemia. Blasts in the peripheral blood had the cytoplasmic protrusions characteristic of megakaryoblasts, others had abundant cytoplasm. No cells could be aspirated from the marrow. A touch preparation of the marrow biopsy included atypical megakaryocytes and many blasts. Sections ofthe hypercellular.marrow revealed decreased erythropoiesis,. many small blasts, large megakaryocytes and micromegakaryocytes. Demonstration of reticulin fibers correlated with the dry tap.

ACUTE MEGAKARYOBLASTIC LEUKEMIA References: Chan ACL et al: Granulocytic sarcoma of magakaryoblastic differentiation ...Hum Path 27: 417, 1996 TTaweek STet al: Extramedullary myeloid cell tumors AJSP 17: 1011, 1993 Roth MJ et al : Extramedullary ~yJ:Ioid cell tumors. Arch Pathol Lab Med 119: 790, 1995 Ashfaq R: Acute megakaryoblastic leukemia. ArnJCiinPath 98: 55,1992

Case 2.(96-R-01710) Invited discussant: Dr. James Strauchen, Mt.. Sinai Medical Center Host discussant: Dr. Elizabeth Ames

Dr. Strauchen pointed out that at low magnification only a portion of the lymph node exhibited abnormalities, consisting of a thickened capsule, proliferating spindle cells I:Yhich grew out of the capsule, obliteration of vesse1s and pleomorphic inflammation including plasma cells. While the differential diagnosis of spindle cell nrmors of lymph POdes and spleen is quite lengthy, it includes only a few hemopoietic tumors, e.g. Hodgkin's disease in HIV may have spindle cells. Vascular transformation of sinuses produces fibrosis of the sinuses with neo\fascularization and with mesenchymill·cells in the stroma The present case lacks hyaline globules and narrow slits, excluding Kaposi's sarcoma .. Castleman's disease of tlie plasma cell type is another possibility as are true hemangiomas. Dr. Rosai described a hemorrhagic spindle-cell tumor of lymph nodes which differs from KS by the presence ofhyalinized collagen and the absence of hyaline globules. follicular dendritic cell tumor, deriving from the major antigen-presenting cell (belonging to the mononuclear phagocyte and immunoregulatory effector system) occurs chietly in lymph nodes, exhibits plump spindle cells with pseudovascular spaces and prominent mixed inflammatory cell infiltrate. Tumor cells stain with CD2l and CD 35. Inflammatory pseudotumor oflymph nodes, first described by Perrone and Frizzera, presents with fever and has a benign course. Dr. Ames informed the audience that the patient had an upper respiratory infection with fever which soon subsided. At a ~x2x2cm . mass adhered to adjacent tissues, muscle, fat and salivary gland. Nonspecific inflammation was noted in part of the lymph node. Collections of foamy histiocytes and proliferating vessels distorted the remainder. Spindle cells and macrophages stained \vitb vimentin. Desmin stairi proved negative, and this is in contrasi to the spindle cell proliteration with amianthoid fibers . Muscle specific actin stained the vessels and CD.68 staining occured in macrophages and spindle cells.

INFLAMMATORY PSEUDOTUMOR OF LYMPH NODE References: Suster S, Rosai J: lntranodal hemorrhagic spindle-cell turnor .. AmJSurgPath 13. 347, 1989 Seijo, Let al : Inflammatory pseudotumor of the spleen. 1m J Surg Path 3: 289, 1996 Perrone T et al : Inflammatory pseudotumor oflymph nodes. Am J Surg Path 12: 35 1, 1988

Case 3 (54104-88 ). Invited discussant: Dr. John Protic. Maimonides Medical Center. Host discussant: Dr. Gaetz ln sections of the RUL cavity from a 35 year old HIV patient, some foci have a clearly granulomatous character. definitely with neutrophils. One may discern faint filaments staining blue \vith the acid fast stain. Silver stain confirms that this is not a fu ngus. and it is Gram positive. Modified acid fast stain. in which colorization is more gentle tha in the regular version, highlights the filaments, which are characteristic of Nocardia. About half the cases ofthis underdiagnosed infection occur in immunocompetent hosts. It responds to treatment for Pneumocystis, and starts as lobar or lobular pneumonia. The organism is astonishingly hardy. and able to grow in such materials as paraffin, jet fuel and testosterone. N. asteroides infects the lung, while N. braziliensis occurs in the skin and subcutis. Dr. Gaetz concurred in this diagnosis and advised that followup information was not available. NOCARDIOSIS References: Rodriguez JL: Pulmonary nocardiosis ...Chest 90: 912, I 986

Case 5. (S-10166-94) Invited discussant: Dr. Helen Feiner. NYU Medical Center Host discussant: Dr. Elizabeth Ames Dr. Feiner demonstrated that the omental biopsy from a 42 year old HIV patient showed plasmacytoid cells with a tendency to form cords, but mostly growing in sheets, with poor cohesiveness between individual cells. Bizarre multinucleated giant cells were found. There was no staining with cytokeratin, CD45, HMB45 and S-1 00. Tumor cells stained withCD3 , BerHI/CD30. and EIVL'\ but not with CD68. The reaction with the two latter antibodies pointed to Ki-1 lymphoma. Only a few cells stained with lambda light chain. CD3 stained the cytoplasm. Dr. Feiner reminded that body cavity lymphomas are mostly null. with only a few cases ofT ~!I type. Immunoglobulin gene rearangement studies identified them as having B cellltneage. These cells have integrated into their nuclei the KS virus (Human Herpesvirus 8). Of 151 HIV patients with lymphoma, eight were cavity lymphomas. A specific feature of such tumors is the absence of myc rearrangement. KS herpesvirus and EB virus are B cell lymphotropic, while HSV (Herpes saimiri) attacks T cells. The collaboration ofKSHV and EBV is related to the development of cavity lymphoma.

Dr. Ames amplified the history, adding that the patient had tense ascites, and nodular densities behind the rectus muscles.Ascitic fluid was cellular, with polymorphous cells, I

multilobated bizarre and huge nuclei, and with basophilic cytoplasm. She felt that the biopsy gave the appearance of immunobiastic lymphoma. Cells failed to stain with both B and T cell markers, but reacted with lamoda and BerH2, as well as with LCA, Ei'vlA and CD20. Heavy chain gene rearrangement was found. KSHV was demonstrated by PCR and Southern blot. BODY CAVITY LYMPHOMA 1N AIDS

References: Green I et al : Primary lym~homatous effusions in AIDS ... Mod Pathol 8:39. 1995 Jones D et al: Cytologic Diagnosis of Primary Serous Lymphoma. AJCP I 06: 359. 1996 Jaffe ES: Primary body cavity-based AIDS-related lymphoma. AJCP 105: 141 , [996 Cesarrnan E et al: Kaposi's sarcoma-associated herpesvirus ... NEJM 332: 1186. \995.

Case 4 (S-10166-94) Invited discussant: Dr. Thomas Wright, Columbia Presbyterian Hospital Host discussant: Dr. Harold Gaetz

Dr. Wright began by noting the nesting, well-demarcated pattern of tumor cells in the ovary, and pointed to the pink cytoplasm. The stroma showed luteinization. There were mitoses, some nucleJii were prominem. ln ptaces the tumor had a tendency to spindle. Alternatives included malignant Brenner tumor (transitional), endometrioid carcinoma, Senoli-Leydig. granulosa cell tumor , luteinized thecoma, and metastatic tumor. Brenner rumor could be excluded because of the ::bsence of transitional cells. papillary folds. and necrosis in the center of nests. Thecoma presents as nodular masses with plump cells and stellate hyalinization. Breast, Gl and endometrial cancers account for 90% of metastases in the ovary and in more than 60% spread is bilateral. Some tumor nests exhibited vacuolated cells with pyknotic nuclei, raising the question of another component. possibly luteinized. The presence of cysts and 'Cellular arrangement suggested Senoli tumor. Tubules wiih cubo.idal and columnar cells are charactenstic of sex cord tumors. Slit spaces point to the ·•rete" type of Senoli-Leydig tumor. Marker stains showed cytokeratin but not in the perinuclear pattern which occurs in gr