.. PATHOLOGISTS` CLUB

101.,6
..
PATHOLOGISTS' CLUB
OPNEWYORK
MEETING
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FUD t. SWJ'IH.WJ).
DATE:
THURSDAY, NOVE'MBER6, 1997
PLACE:
BELLEVUE/NYU MEDICAL CENTER
560 FIRST AVENUE
NEW YORK, NY 10016
HOST:
JAISHREE JAGIRDAR, MD
INFORMATION:
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SCIENTIFIC
SESSION:
CLASSROOM S
7:00- 9:00PM
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The Faculty Dining Room is on the ground floor of Schwartz Health Care Center at NYU Medical
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The scientific program will be held in Alumni Hall, Classroom B. Enter the Medical Center
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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.
Case #2
BS97-953
HP97-623
Invited Discussant: Howard Ratecb, MD
Albert Einstein Medical Center
Host Discussant:
Glauco frizzera, MD
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.
Case #3
Invited Discussant: Harry 1.. Joachim, MD
l.cnox Hill Hospital
Host Discussant:
Susan Kornacki, MD
The patient was a critically ill fourteen year old male with AIDS. CD4=0. A Chest CT showed ground alass 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.
Case#4
S97-3639 (I K)
Invited Discussant: Maria Luisa Carcingiu, MD
Yale New Haven Hospital
Host Discussant:
Khush MiHal, MD
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.. wsalpi.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.
Case#S
Invited Discussant: George M. Kleinman, MD
Bridgeport Hospital
Host Discussant:
David Zagzag, MD
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.
PATHOLOGISTS' CLUB
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November 6, 1997
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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 . Th e 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 ha d . 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 ll orna~os is ( FP), clear c e ll change
i n the eoid erm1s, 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 typ1ca l c ells to reside in the
l actiferous ducts and not i n ~he s kin. Proliferation o f tubu l es
suggests FP. Other possibil i t i es to consider: Intraductal
pap1 lloma ( but this lacks a fi brovascular core, so it is
e xcluded), Paget's, syringomatous adenoma (but this i s a tumor of
skin) and i n-situ a na invasive c arc i noma . Absence of s evere
celluiar atypia and proliferation ·of myoepithe lial 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
the re 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
re ported 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 c ells , in c ontrast 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 res embl 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.Ramacha ndra s et a l: A comparative i mmunohistochemica l study of
mammary and e xtramammary .. vi rchows Arc hiv 429 : 371 ,
1996
3.Vi anna LL et al: Adenoma of the n i pple .. BrJ HospMed 5 0 : 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 . 5c m nodule . Ra r e giant
c e l l s at presentat ion were t hought to indicate herpe svirus
i nfec tion.
Guest discussant: Dr. H. Ra tech , Montefiore Hed. Center
Host: Dr . G. Fr izzera
The nodula r infi ltrate in the s uoerficia l and d eep dermis i ncludes
plasma cells, lymphocytes, e osinoohils and l a rge cells resembling
J.mmunoblasts . !nfiltr a'C.i on of hair"follicle epithe lium b y a cell
mixture i ncludi ng neutrop hi ls s uggests heroes infec tion. Antibodr
s ta i ns demonstrate herpes, B cel ls (L26 ) a nd more numerous T eel s
(UCHL- 1) . Lar ge c e lls a ppear to s t al.n more we akly; they are Kl
nega t ive , CD30 oos i~ive, and show mitoses . Different i al i ncl udes T
c ell l ymphoma in AIDS , which seems t o be ~he d iagnos is here ,
a naplastlC large c ell lymphoma, l ymphomatoid papulosis, pseudolyrnehoma a nd Hodgkin 's. Cutaneous present ation of lvmphoma take s 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
f ew of the l a rge r 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
g ene)in both . This find J.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
c onsidered ne oplas tic . Fa lowing the meeting , Dr. Frlzzera k ind l y
a mplified t he aiscuss ion , e mpha s i z ing t hat , as i n other s ki n
~ iseases , s~ch a ~ l~phoma to1d 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 te rm l ymphoma. The i mplic ation a t
this s tage i s close f ollow-up rather than a ntineoplastic the rapy .
All fi ve biopsies taken look similar.
·
Dx: Atypical l ymphoid i nfil t race in an HI V pos itive man
1 . Kersc hmann et al : Cut aneous presentat ion of lymphoma .. Arc hDerrn
131: 128J. ' 1995
2 .Macgroga~ G et al: CD3o-posi tive c utane ous large cel l lymphomas.
AmJCllnPathl05:440 , 1996
3 . Knowles OM et a l: 11ole cular genetic a nalysis .. Blood 7 3:792, 1989
4 . Crane GA e~ al : Cut a neous Tcell l ymphoma . ArchDe r m 127 : 989, 1991
Case 3. A c ritica lly ill 14 year o ld boy with AIDS, CD4 of ze ro and
ground glass opacit1es on chest CT did not respond to empiric
t hera py for Pneumocys tis pneumonia. He d i ed two days late r and was
autopsJ.ed by the Meaical Exami ner' s s taff because of s uspected
chila a buse.
Gues t Disc ussant: 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
pneumocyte s, some i nt ersti tial 1nfiltrate ·congestion ana
hemorrhage . The r e a re small basophilic intra nuclear inc lusions
which are not condens ed. Both proximal convoluted and c o l l ecti ng
tubu l es o f the k idney show obv1ouslY virus-infected cel ls . Lac k o f
homog enei t y in f orm s ugges ts it i s not a c ommonly found v irus .
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.
In vestigat1on 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 a ntibody stains are negati ve for adeno
virus. and RSV .Necrotizing c hanqes wh1ch are expected with
adenovirus and Cl1V are not seen. Polyclonal antibody stain for SV40
cross-reacts wi th polyoma v irus , 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 hastened 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 : D1sse minated 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, a lso had extensive
metastases i n the f allopi an tuoes, uter1ne serosa , cerv ix and
vag i 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 wi th 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 e llular stroma
with prol~ fer<;!.tion of the same c ells 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 c omponent . The question i s i f i t i s primary or
metasta.t ic . P rimary mucinous carcinoid of the ovary was described
by Talerman ( 1986 ) . lfistor.icall v a metastatic gastrointestinal
mu·c inous 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 hypercell ular
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 endoc rine
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 % (primaril y signet rina and
mixe d 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 d ue 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~lPatho 1 4 : 110, 1985
Case 5 . 54 year old man wi~h right oarie~al headaches . fatigue
somno l e nce and vomiting . He has no sensory deficit, norma l tendon
reflexes external, ocular noveme nts and v1sual fields , bu~ mild
left uooer ex~rem1tv d ri ft . MRI reveal s a 6cm right f ron~al durabased tumor, a lcm left Heckel's c avitv tumor ana a !.Scm riaht
cavernous s1nus tumo r. Section is trom-~he fron~al tumor.
•
Guest discus sant : Dr. G. r1.Kleinman, Br1 dge port 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, lePtomeninge al
soace and c erebrum. 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 a nd a mixed i n flamma~ory cell
i nfiltrate are noted . Differential consists of malignant melanoma,
anaolastic large c el l lymphoma and mal ignant mening1oma. Tumor
cells sta1n Wlth v iment~n , £MA and s - 100, bu~ not with AE1/AE3 , CAM
5 . 2 a nd GFAP. Ce ll junctions are f ound 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 ·.
. JNeu ro l Neur~surgPsyc n 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 c entr al ner vous
System . . JNeuropathExpN.e urol 50 : 362, 1991
5 . Aki mura T e t a l: Malignant men~ngi oma .. ActaNeurScand 85 : 368,1992