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DIRECTIONS:

PATHOLOGISTS' CLUB OPNEWYORK

MEETING

DATE: THURSDAY, NOVE'MBER6, 1997

PLACE: BELLEVUE/NYU MEDICAL CENTER 560 FIRST A VENUE NEW YORK, NY 10016

HOST: JAISHREE JAGIRDAR, MD

INFORMATION: MARlJ~VNROSADO {212) 263-6445

RECEPTION AND 5:15-6:45 PM DlNNER: FACULTY DINING ROOM

SCIENTIFIC SESSION:

CLASSROOM S 7:00- 9:00PM

The Faculty Dining Room is on the ground floor of Schwartz Health Care Center at NYU Medical Center. Enter the Medical Center through the Main Lobby on First Avenue, between 31" and 32"" Streets.

The scientific program will be held in Alumni Hall, Classroom B. Enter the Medical Center through the main lobby on First Avenue, between 31" and 32ncl Streets.

New York University Medical Center can be ;·:::ached by #MIS buses, which run north on I" Avenue and south on 2nc1 Avenue, and by #Ml6 buses, which run crosstown on 34m St. The nearest stop of a subway is at Park Avenue and 33'd St {theiRT Lexington Avenue local train, #6). Commercial public parking is available at the Kips Bay parking lot on I" Avenue (west side) near J2nd St., and on the north side of29111 St. between I" and 2oc1 Avenues.

THE NEXT MEETING WILL BE HELD AT MONTEFIORE HOSPITAL ON DECEMBER 4TII.

i

Case #1 Invited Discu$$4Jit: Hanina Hibshoosh, MD

Columbia College of Physicians and Surgeons

Host Discussant: W. F. Symmans, MD

The patient is a 43 ye:.s old white woman with excoriation of the left nipple. No underlyi.n& palpable mass was present. The patient had a nipple scraping followed by a punch biopsy, and a wedge resection of the nipple. A mammogram was negative. The kodachromes are from the scraping and from the wedge ~escction.

BS97-953 HP97-623

Invited Discussant: Howard Ratecb, MD Albert Einstein Medical Center

Host Discussant: Glauco frizzera, MD

Case #2

The patient is a 52 year old HN-positive male with a CD4 count of IS, who had a left buttock non-healing ulcer which developed into a 4.5 x 4.3 em nodule, over a period of 1 year. At the rime of presentation, the ulcer contained some rare multinucleated giant cells, consistent with a Herpes Simplex infection. The slides are from the recent nodule.

Invited Discussant: Harry 1.. Joachim, MD l.cnox Hill Hospital

Host Discussant: Susan Kornacki, MD

Case #3

The patient was a critically ill fourteen year old male with AIDS. CD4=0. A Chest CT showed ground a lass opacities. A clinical diagnosis of PCP was made. However, he did nor respond to empiric therapy for PCP. A diagnosis of lymphocytic inremitial pneumonitis was then suspected, but could nor be subslllnriated. The patient died two days later, and an autopsy was performed at the Medical Examiner's Office, because the youngster was suspected to be a victim of child abuse.

S97-3639 (I K)

Invited Discussant: Maria Luisa Carcingiu, MD Yale New Haven Hospital

Host Discussant: Khush MiHal, MD

Case#4

The patient is aS I year old female, who presented with an abdomino: mass. Bilateral ovarian enlargement was noted ( 12 x 6 x 5 ern and 7..5 x 5 x 4 em). A total abdominal h:r.terectorny wnh bilar .. w salpi.nguopborectomy was performed. Both ovaries were enlarged and had a bosselated surface. On cut section, the ovaries were replaced by a variegated soft, tan and partially hemorrhagic tumor. Both fallopian tubes, the endometrium. uterine serosa. cervix and vagina were extensively involved by the IUmor on microscopic exDmination. The microscopic appearance or the tumor in these oraans was similar (0 that of the ovary. A scerion of !he left ovary is provided.

Invited Discussant: George M. Kleinman, MD Bridgeport Hospital

Host Discussant: David Zagzag, MD

Case#S

The patient is a 54 year old male with one monlh history of right parie~al headaches and more ruenr vomiting. The headaches were worse at night and in the moming. The patient complained of fatigue and somnolence. Physical examination revealed an oriented and fully awake patient. Neurological examination showed mild left upper extremity drift with normal external ocular movements and visual fieJds. There was no sensory deficit, and the tendon reflexes were normaL Magnetic resonance imaging showed a 6 ern right frontol dural-based rumor, a 1 ern left Meckel cave tumor, and a I.S ern right cavernous sinus rumor. The frontal rumor was removed.

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PATHOLOGISTS' CLUB 01" NEW YORK

lHNUTES OF MEETING November 6, 1997

BELLEVUE HOSPITAL

An excellent sc1entif i c program organ1zed by Dr . Jagi rdar and with the participation of some of the region' s outstanding patholog1s~s attracted a large ana en~hus:;.astic audl.ence . The Cl ub membership welcomed t wo new members, Dr . Dr.Muhammadsamir Sul h of Bronx Lebanon Hospital

a nd Dr . Olcay Dirnopulo of Bellevue Hospita l.

case 1 .43 year o l d woman with excoria~ion of the L. nipple but without other ohvsical and mammographic f i ndings had . a scrap1ng fol lowed by punch b1opsy and wedge resec~1on .

Guest discussant: Dr. H. Hibshooosh, Columbia P&S Host: Dr . W.F . Symrnans

The differential d iagnos1s o f Page~ ' s disease incl udes i nfla m­mation (eczema), florid paoi llorna~os is (FP), clear cell change i n the eoiderm1s, melanoma: s auamous a nd basal cell c ar cinoma,and s yringoma . The scrape preparati on shows atypical cel l s . The excised speci men rev€als the a typ1cal c ells to reside in the l actiferous ducts and not i n ~he s kin. Proliferation of tubul es suggests FP. Other possibil i t i es to consider: Intraductal pap1 lloma (but this lacks a fi brovascular core, so it is excluded), Paget's, syringomatous adenoma (but this i s a tumor of skin) and i n-situ ana invasive carci noma . Absence of severe celluiar atypia and proliferation ·of myoepithelial cells point away from carcinoma . SHA stain suggests proliferation but not invas ive tumor . FP may arise in any age and presents with bloody discharge , pai n and itching. Most cases have a mixed pattern , but there may occur a pura adencsis, panillomatosis or sclerosing papillomatosis. In-situ and invas1ve carcinoma coexist in 16% of cases of FP . Carcinoma arising i n FP of the nipple has been reported only i n eight patients. The atypical cel ls in the ep1dermis stain with CAM 5 . 2 but they lack malignant f eatures, therefore they must belong to the proliferating epi thelium emerging from t he ducts onto the surface. Dr. Symmans noted that t he pat1ent has a sister i n her late 30's with breast cancer. In the smears some atypical cells occur in clusters and in association wi t h myoepithelial cells , in contrast to Paget's disease which presents large , obviously malignant cells . Within the FP in the s ections there i s a focus t hat closely resembl es carcinoma. Cells staini ng wi th SMA enclose every group of suspicious cells . CAM 5.2 staining is found in both Paget's cells and in duct epithelium, thus t his is not helpful. Wedge resection was f e l t to be adequate treatment. Dr . Rosai pointed out that Toker described c lear cells in the nipple epidermis in patients wi thout clinical Paget' s a nd wi thout breast carcinoma . These are ductal cells and may show slight atypia.

Ox: Florid papillomatosis of nippl e

l.Mirka WJ et al: coexistence of nipple duct .. HodPath 8:633 , 1995 2.Ramachandra s et al: A comparative i mmunohistochemica l study of

mammary and extramammary .. virchows Arc hiv 429 : 371 , 1996

3.Vi anna LL et al: Adenoma of the ni pple .. BrJ HospMed 50 : 63 9, 1993 4. Toker c : Clear c ells of the nipple .. Cancer 25 : 601 , 1970

Case 2. 52 year old HIV positive man with CD4 of 15 had a buttock non-healing ulcer whi ch progressed t o a 4 . 5cm nodule . Rar e giant c e l l s at presentat ion were t hought to indicate herpesvirus i nfection.

Guest discussant: Dr. H. Ratech , Montefiore Hed. Center Host: Dr . G. Fr izzera

The nodular infi ltrate in the s uoerficia l and deep dermis i ncludes plasma cells, lymphocytes, eosinoohils and l a rge cells resembling J.mmunoblasts . !nfiltr a'C.i on of hair"follicle epithe lium by a cell mixture i ncludi ng neutrophi ls suggests heroes infection. Antibodr s tai ns demonstrate herpes, B cel ls (L26 ) a nd more numerous T eel s (UCHL- 1) . Lar ge c ells a ppear to s t al.n more weakly; they are Kl nega t ive , CD30 oosi~ive, and show mitoses . Different i al i ncludes T cell l ymphoma in AIDS , which seems t o be ~he d iagnos is here , anaplastlC large c ell lymphoma, l ymphomatoid papulosis, pseudolyrn­ehoma and Hodgkin 's. Cutaneous present ation of lvmphoma takes two rorms, e ither as nyyo9is fung9iaes or non~piderm9tropic, with 80% of t he l atter contalnlng EB v1rus. Dr . Fr1zzera lntroduced Dr . I nghirami who completed t he discussion. He finds t hat relat ively few of the l a rger cells are ofT type (CD3 positive) . Employing PCR to check clonalitv in two biopsies of t he same ski n site he r epor ts a s mall rearr angement band of the a ammaTCR (T cei l receptor gene)in both . This findJ.nl and s trong sta1ning f o r EB virus prove that a clonal population EB driven) is diss eminating a nd is to be considered neoplas tic . Fa lowing the meeting , Dr. Frlzzera kindl y amplified t he aiscuss ion , e mphas i zing t hat , as i n other skin ~iseases , s~ch a~ l~phomato1d papulos is and ~ycosis fungoi des, the lnt eroret atlon or this case does not necessar1lv carr v an aggress­ive connotat ion, as would t he term l ymphoma. The i mplication a t this s tage i s close f ollow-up rather than antineoplastic therapy . All fi ve biopsies taken look similar. ·

Dx: Atypical l ymphoid i nfil t race in an HI V pos itive man

1 . Kerschmann et al : Cut aneous presentat ion of lymphoma .. ArchDerrn 131: 128J. ' 1995

2 .Macgroga~ G et al: CD3o-posi tive cutaneous large cel l lymphomas. AmJCllnPathl05:440 , 1996

3 . Knowles OM et a l: 11olecular genetic analysis .. Blood 7 3:792, 1989 4 . Crane GA e~ al : Cut aneous Tcell l ymphoma . ArchDer m 127 :989, 1991

Case 3. A critica lly ill 14 year old boy with AIDS, CD4 of zero and ground glass opacit1es on chest CT did not respond to empiric t herapy for Pneumocys tis pneumonia. He d i ed two days late r and was autopsJ.ed by the Meaical Examiner' s s taff because of suspected chila abuse.

Guest Discussant: Dr. H. L. Ioac him, Lenox Hill Hospital Host: Dr. J agirdar

Lung secti on shows pale- s taini ng alveolar exudate, hyperplast i c pneumocytes, some i nt ersti tial 1nfiltrate ·congestion ana hemorrhage . Ther e a re small basophilic intranuclear inc lusions which are not condens ed. Both proximal convoluted and co l l ecti ng tubul es of the kidney show obv1ouslY virus-infected cel ls . Lack of homogenei t y in form s uggests it i s not a commonly found virus .

Adenovirus exhibits Cowdry A inclusions, with smudged cells in lung , kidney, liver_ and pancreas, with necrosis and with diffuse alveolar damage. Ultrastructural study reveals arrays of icosahe­dral forms, cnaracteristic but not diagnostic of adenovirus. Investigat1on by in-situ hybridi zation did not support either adenovir us or respiratory syncytial virus (RSV). other viruses with icosahedral symmetry include garvovirus, enteroviruses, polyoma and herpes (HSV 1 EBV, CMV). Size is the most important determinant. Whil e morpholog1cally this i s c l osest to adenov1rus 1 its size of 35nm is agains~ it. Polyoma (BK) has been reported ~o cause hemorrhag1c cystitis in a llograft patients , and more recently to cause DAD, interstitial pneumoni a , tubulonephritis and meningoencephalitis. Dr. I oachim concludes that this is probably adenovirus,·with polyoma ( BK ) as the second choice. Dr. Jagirdar also finds that antibody stains are negati ve for adeno virus. and RSV .Necrotizing chanqes wh1ch are expected with adenovirus and Cl1V are not seen. Polyclonal antibody stain for SV40 cross-reacts wi th polyoma virus , and in addition to nuclear there is some cytoplasmi c staining. This non-enveloped DNA virus i s apout 45-55nm in spe. Rapid progression of diffuse alveolar damage hast-ened the pat1ent' s _a_eatb . . ·

Ox : Polyoma virus i nfection

l .Pappo 0 et a l : Human polyoma virus . . Mod ~ath 9: 105, 1996 2. Vallbracht A et a l : D1sseminated BK t ype . . AmJPath 143 : 29, 1993

Case 4 . 51 year old ~loman with both ovaries replaced by variegated soft, tan and f ocally hemorrhagic tumor, also had extensive metastases i n the f allopi an tuoes, uter1ne serosa , cerv ix and vagi na .

Guest d iscussant: Dr. t1 . L. Car cangfu, Yale New Haven Jlospi tal

Host: Dr . K. :Mfttal

ovarian tumor sections show edematous s troma with more cellular f oci. There are nests of sianet r ing c ell s and tubules lined by eosinophilic granular cells~ At the edges there is c ellular stroma with prol~fer<;!.tion of the same cells and l ymphati c inVasion. Tumor cells sta1n Wlth muc1n, CK20 and CEA, and only very rare cell s show chromograni n . Therefore, this is a muci nous adenocarcinoma with a minor endocrine component . The question i s i f i t i s primary or metasta.tic . Primary mucinous carcinoid of the ovary was described by Talerman ( 1986 ) . lfistor.icall v a metastatic gastrointestinal mu·cinous tumor is known as a Krukenberg tumor: unfortunatel y over the years its definition has lost clar1ty as various writers have appl1ed it to tumors from sit es other ~han the GI tract. In a Krukenbero tumor the stroma can be so hypercellular that the carcinoma cells are obscured, leading to a diagnosis of fibrosarcoma. Scully (1981) compared ~ubular Krukenberg with sertoli cell tumors . . Mucin stain. is useful in excluding lipid cell (Sertoli)tumors. Breast, stomach and pancreas are the most frequent sources of Krukenberg tumor . Of 13 cases of tubular Krukenberg tumor, the primary was not determined i n seven. More recently the appendix is reported as a frequent primary site and this is possible in th1s case . Pr imary mucinous carcinoma wi th endocrine features can also spread from the endometrium, cervix, bladder, and biliary tract. Thus, Dr . carcangiu concludes that this a Krukenberg tumor (muc1nous carcinoma with endocri ne features)~ probaol y metastatic. Dr . Mittal notes that i n 1988 the patient unaerwent resection of the appendix , right hemicolon and regional lymph nodes for carcinoid of the appendix which was compress1ng the cecum. El ectron dense granules and chromogranin staining are found in the ovarian tumors. Additional support derives from staining with both CK7 and CK20.

In the most recent studv, about 50 % (primarily signet rina and mixed cell tvoe ) of aooemdiceal adenocarcinomas stain witfi CK7. colorectal appendiceal·and primary ovar ian mucinous carcinomas near ly always s~ain wi th CK20.Therefore, wh~n th~ morphology of a muc~nous tumor 1n the ovary is cons1stent w1th e~ther ovar~an or appe~diceal primary, stain1ng for CK7 does not suppor t ovar ian or1g1n.

ox: Mixed.carcinoid-adenocarcinoma of appendix metastatic to ovar1es

1 . Alenghat E e t al : Primary mucinous carcinoid tumor .. Cancer 58 :777,1986

2.Ronnett BM e t al : The mor phologic spectrum .. AmJSurgPath 21 :1144 1997

J . Klein EA et ai: Bilateral Krukenberg's t umors due to aopendiceal .. IntJGvneco1Pathol 15 : 85, 1996

4.Burke A~ et al: Goblet cell carcinoids .. AmJClinPath 94 :27 , 1990 S.Her1no t-IJ e~ al: Appendiceal carcinoma metast atic to the

ovaries .. Int Jgynec~lPatho1 4 : 110, 1985

Case 5 . 54 year old man wi~h right oarie~al headaches . fatigue somnol ence and vomiting . He has no sensory deficit, norma l tendon reflexes external, ocular novements and v1sual fields , bu~ mild left uooer ex~rem1tv dri ft . MRI reveal s a 6cm right fron~al dura­based tumor, a lcm left Heckel's cavitv tumor ana a !.Scm riaht cavernous s1nus tumor. Section is trom-~he fron~al tumor. •

Guest discus sant : Dr. G. r1.Kleinman, Br1dgeport Hospital Hos~. Dr. D. Zagzag

The cavernous sinus mass mav be connect ed t o the f rontal tumor. section shows necrosis, wic~ invasion of the dura, lePtomeningeal soace and cerebrum. In olaces the 'tumor lS papillary, · raising -che q\Je~tion ~f •.~he~her ~ t is truly a . papillary m~ningiom<;1 or only a m1m1c . I n~ranuclear 1nclus1ons po1n~ ~o a menlngothellomatous tumor or a malignanc melanoma. Giant cells and a mixed inflamma~ory cell i nfiltrate are noted . Differential consists of malignant melanoma, anaolastic large cel l lymphoma and mal ignant mening1oma. Tumor cells sta1n Wlth viment~n , £MA and s - 100, bu~ not with AE1/AE3 , CAM 5 . 2 and GFAP. Cell junctions are found on EM. The patient also had cysts in the liver and kidney and developed a left III nerve palsy and enlarqemen~ of tumor i n the l eft i nteroeduncular f ossa .

ox : Maligna nt men ingioma w1~h r habdoi d features

!.Ludwin SK et al: Ma lignc-. r-+- !!'e.r> insicl!'a rnet.actasiZil•9 ·. . JNeurolNeur~surgPsycn 38 : 136, 1975

2. Kobayash1 C et al: Men1ngeal rhabdomyosarcoma . . Acta cytol 39: 428, 1995

3 . Ferracini R: Meningeal sarcoma . .. Neurosurg 30: 782, 1992 4. Kepes J J et al: Malignant rhabdoid tumors of the centr al ner vous

System . . JNeuropathExpN.eurol 50 : 362, 1991 5 . Aki mura T e t a l: Malignant men~ngioma .. ActaNeurScand 85 : 368,1992