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GASTROINTESTINAL TRACT PATHOLOGY

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					                                                        IIC,J
       CALIFORNIA
       TUMOR TISSUE REGISTRY




"GASTROINTESTINAL TRACT PATHOLOGY"

           Study Cases, Subscription A

                      March 2000




             California Tumor Tissue Registry
       c/o: De1mrtment of Pathology and Human Anatomy
            Loma Linda Univcr.;ily School ofMcd.icine
                11021 Campus Avenue, AH 335
                 Lomn Linda, California 92350
                        (909) 558-4788
                     FAX: (909) 558·0188
                    E-mail: cttr@linklhie.com
          Case oftbe Month: www.llu.edu/Uu/cttr/cotm
Target audience:
 Practicing pathologists and pathology residents.

Goal:
 To acquairu the participam with the hiswlogic features of a variety of benign
    and malignant neoplasms and tumor-l ike conditions.

Objectives:
 n1e participant will be able to recognize morphologic features of a variety of
    benign and malignam neoplasms and tWllOr-like conditions and relate
    those processes to pertinent references in d1e medical literature.

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

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

CME Credit:
 Lorna Li.nda University School of Medicine designates this continuing medical
    education·activity for up to 2 hours of Category I of the Physician's
    Recogn ition Award of the· American Medical Association.
 CME credit is offered for d1e subscription year only.

Accreditation:
 Loma Linda University School of Medicine is accredited by the Accreditation
    Council for Continuing Medical Education (ACCME) to sponsor
    continuing medical education for physicians.
Contributor: James A. Henry, M.D.                             Case No. 1 - March 2000
             Woodbridge, VA

Tissue from: Terminal ileum                                   Accession #28502

Clinical Abstract:
       This 37-year-old black female presented with several weeks' history of right lower
quadrant abdominal pain radiating to the right side of the back and right inguinal area. There
was no associated fever, leukocytosis, nausea, vomiting or diarrhea. A pre op CT scan revealed
a 6.0 em so lid mass of t he lower quadran!. A laparotomy with a partial bowe l resect ion was
performed.

Gross Pathology:
       The 11.0 em portion o f ileum inc luded a4.0 em portion of cecum, the appendix and
attached mesenteries for an overall dimension of 18.0 x 18.0 x 7.0 em. The ileal mesentery
contained a 9.0 x 6.0 x 4.5 em friable yellow-tan mass and several separate enlarged lymph
node~ measuring up to 2.0 em in greatest dimension. The appendix was adherent to this mass.




Contributor: Mark Lones, M.D.                                 Case No. 2. - M:trch 2000
             Ornnge, CA

Tissue from : Rectum                                          Accession #28148

Clinical Abstract:
       Whi le being evaluated for rectal bleeding, thi s 73-year-old male patient was found to
have a 2-3 em firm area i.o a thickened rectal wall. l:ollowing a co looscopic biopsy. an
abdominal perineal resection of the rectum was perfom1ed.

Gross J>atholo!!Y:
        In the mid portion of a 39 em length of bowe l WclS a 3.0 x 1.5 x. 0.8 em centrally ulcerated
red-tan tumor. The mass grossly extended into and through the bowel wall.

SPECIAL STAINS (o utside      facil ity):
     Chromogranin              focal positivity
     Synaptophysin             focal positivity
     CAM 5.2                   diffusely positive
Contributor : Philip G. Robinso n, M.D.                      Case No. 3 - Mar ch 2000
              Boynton Beach, FL

Tissue from: Small bowel                                     Accession #284 79

Clinical A bstract:
         At surgery for an acute abdomen, a nodule was nOled in the bowel o f th is 43-year-old
fe male.

Gross P athologv:
        A segment of small bowel showed a 4.5 x 2.8 x 3.2 em nodule projecting into the lumen.
It had a light yellow to tan cut sur lace and extended to the serosa.




Contributor: J im Han nah, M.D.                              Case No.4 - Mar ch 2000
             San Luis Obispo, CA

Tissue from : Colon nod small bowel                          Accession #28153

Clin ica l Abstr act:
        This 36-year-old male presented with abdominal fullness. An exploratory laparotomy
found a multiloculated cystic mass invo lving the serosal surface o f the colon a nd small bowel.

Gross P athology:
       The tumor was approximately 20 em in greatest dimension and appeared to be multifocal.
Contributor: Farooq Ali, M.D.                                 Case No. 5 - March 2000
             Vcntum, CA 93003

Tissue from: Appendix                                         Accession #28680

Clinical Abstract:
        Because of complaints of right sided flank pain. this 86-year-old Hispanic female bad a
CT scan, which s howed a simple cyst of the right kidney and a 2 e m cyst in the right lobe of t he
liver. About two years later, she presented with repeated episodes of right sided pain.
Radiographic studies showed the cysts to be unchanged but gal lstones were noted and she
underwent a cholecystectomy. At that time, a perforated appendix was found, with multiple
adhesions and mucus-filled cysts confined to the append ix and mesoappendix. Ovaries were
norma l ror age.                        -

Gross P:llhology:
       The 5 em long appendi x was up to 1.5 em in external diameter. It had hemorrhagic red
adhesions and multiple mucin-ti lled cysts up to 1.5 em in diameter on the mesothe lia l surface.




Contribu tor: Fr.tncis S. Buck, M.D.                          Cas e No.6 - March 2000
              Los Angele.s, CA

Tissue from: Liver                                            Accession #17251

Clinical Abstract:
        A live month girl failed to thrive, developed chronic anemia and hydrocephalus, and then
expired in her filth monlh o f life. She weighed 2 po unds 4 ounces at birth and measured 38 em.
Both the mother and baby were 0, Rh positive. The child received exchange transfusions on her
third and fourth day of life because of hyperbilirubinemia of undetermined etiology.

Gross Pathology (Autopsy):
      The liver weighed I 02.5 grams and was yellow-tan and contained many small. pearl-like.
,-ay-white tumors measuring up to 0.4 em in diameter.
b

                                                                                     I
Contributor: Tom Schmidtkecht, M.D.                          Case No. 7 - March 2000
             Oakland, CA

Tissue from: Abdomen                                         Accession #26302

Clinical A h~trnct:
        During a hysterectomy fo r mu ltiple leiomyo mas, a large pcdw1culatcd hepatic moss was
noted o n the inferior surface of the liver ofthis 49-year-old fe male. Other simi lar appearing
masses were noted within the hepatic parenchyma but were not removed.

Gross Pathology:
        T he 174 gram mass was 10.0 x 9.0 x 4.0 em. Sectioning revealed a spongy red-purple
tissue with scattered gray-tan nodules measuring up to I. 7 em.




Contri butor: LL Pathology Group (drc)               Case No. 8 - March 2000
              Loma Lin da, CA

Tissue from: Liver                                   Accession #28489

C linical Abstr.t ct:
        Du ring a routine phys ical exam ination, a large mass was found in the upper abdominal
quadrant of this 58-year-old temalc. Questioning revea led that she had experienced a 70 lb
weight loss over the past two years. CT of the abdo men s howed a soft tissue de nsity posterior to
the stomach adjacent to the pancreas.

Gross Patholo gy:
       The2480 gram. 30.0 x 19.0 x l i.Ocm spccimen co nsisted ofa 17.0 x 1     5.0 x 10.0 linn
multinodular tan encapsu lated tumor with attached distal pan.creas, spleen and a segmcm of
colon. There was no gross invasion of these structures by the tumor. The cut surface of the
tumor was firm, tan, whorled and nodular.
Contributor: David Lawrence, M .O.                          Case No. 9 - March 2000
             Santa Maria, CA

Tissue from : Rigbt liver                                   Accession #28553

Clinica l Abstract:
       This 82-year-old female presented with a right liver mass, which was resected.

G ross Pathology:
       T he 6.0 em mass had numerous sate ll ite lesions extending over an additiona15.0 cm area.




Contributor : Isabel Salcedo, M.D.                          Case No. 10 - M arch 2000
              Fontan a, CA

Tissue from: Pa ncr eas                                     Accession #26335

Clinical Abstr-.tct:
       After complaining ofimem1ittcnt epigastric pain for two months. tllis 39-year-old fema le
was found to have a cyst ic mass arising from the body of the pancreas. At surgery, a thin-
walled, mucin-filled mass was attached to the pancreas and pushed tbe stomach to the rigbt.

G ross Pathology:
       The 850 gram. multilocular cystic mass was 16.0 x 12.0 x I 0.0 em.
                           CALIFORNIA
                           T UMOR TISSUE REGISTRY



                                      GASTROINTESTINAL TRACT PATHOLOGY

                                                      Minutes- Subscription A

                                                                 March 2000



                         SUGGESTED READING (General Topic.1 fro m Recent Literature):

Redefining the Role of Antiarrhythmic Drugs. P(>drid PJ, et al. The New England Journal ofMedicine 1999;
   340(24):1910- 1911.
tumor Suppressor Genes. A Short Review. Yeo, CJ. Surge1y 1999; 125(4):363-366,
Post-Tral)splant Lymphoprolifetative Disorder. A Practical Approach. Swerdlow SH. Scm Diag Patlwl 1997; 14:2,
Is Intensive Follow-Up Really Able to Improve Prognosis of Patients with Local Rccun·ence after Curative Surgery tbr
    Rectal Cancer? Secco GB, et al, Annals ofSurg Oneal '1999; 7(1):32-37.
Neurologic complications of the Reactivahon of Varicella-Zoster Virus. Gilden DH, et al. New England Joumtrl of
    Medicine 2000; 342(9):635-645.
Diagnostic Problems in Surgical Pathology of the Adrenal Glands. Lack EE, et al. Mod Pathol 1995: 8(3):3 12-33;!,




                                      California Tumor Tissue Registry
                             c/o: Department of Patholo&,'Y and Human Anatomy
                                  Lorna Linda lJniversity School of Medicine
                                       l )(l21 Campus Avenue, AH 335
                                        Loma Linda, California 92350
                                                (909) 558-4.788
                                               FAX: (909) 558-0188
                                              E-mail: cttr@linkline com
                                  Case oftl1e Month: www. llu.edu/llu/cttrlcotm
Case No. 1, Accession No. 28502                                                                  1\>IR n:b 2000

~rsfield    - Inflammatory pscudotumor (x1mthogranuloma)
Riverside - Inflammatory pseudotumor
Mountain View (EJ Camino Hosl!iW) - Rosai-Dorfinan disease
Qrange <UC! Medical Center Residents) - Extranodal sinus histiocytosis with massive lymphadenopathy (5),
    Inflammatory pscudotumor (3)
tlayward - Organizing periappcndiceal abscess (1)
Vwtura CUnilabl - Crohn's disease (2)
Santa Rosa - Rosai-Dorfinan disease (I); Probable Rosai-Dorfman disease, rule out specific inflammatory reaaion (2)
Santa Barbara (Cottag~ Hospila!l - Sinus histiocytosis with massive lymphadenopathy. extranodal
Monterey (Community Hostlil.al of Monten;y Pcojosula) - Sinus histiocytosis with massive lymphadenopathy
San Die<to CNayal Medical Cenjer} - Rosai Dorfman
Long Beach - Rosai-Dorfman disease (6)
Michi~"'" (Dearborn) - Sinus histiocytosis with massive lymphadenopathy
Texas Ciexas Tech Medical Health Center) - Chronic inflammatory reaction with hemophagocytosis rule out
   amebiasis
Nebra.<ka CCreigb(on University) - Xanthogranuloma
Florida (\<!onroe Regional Medical Center) - Rosai-Dorfinan disease
Marvlaod (University ofMarvlnndl - Rosai-Dorfman disease (extra nodal sinus histiocytosis with massive
    lymph ad en(> pat by)
Ma> :yland !Wooslbincl - Rosai-Dorfman diseuse (2)
Maryland <National Naval Medical Cented - Inflammatory pseudotumor (6). Ycrsinia emercx:olitis (2)
New Jersey (Qver!ool; Hospi1all - Inflammatory pseudotumor (3)
Pennsylvania (Lehigh Valley H.Qlipitall - E.xtranodal Rosai-Dorfrnan disease
l'ennsylvania <Conemaugh McmorialffQspit!tll - Malakoplakia
New York !Monleliore Medical Center) - l!xtranodal Rosai-Dorfinan disease
~ow York (Long Island Jewish Medical Cemer) - Rosai-Dorfinan disease
Connecticu! l!lnjyersitv of Connecticut Heahh Center) - Rosai-Dorfinan disease
Canada CFoothills Hospital) - Mesenteritis (sclerosing)
l.nnan (Shimada City! - Sclerosing mesenteritis
Australia CSydneyl - Sinus histiocytosis with massive lymphadenopathy


DIAGNOSIS:
     E:d ranodnl Rosai-Dorfman Dist'nse, terminal ileurn
                   T-65520, M-778 10

CQNSULIAT10N: Dr. Elaine Jaffe, National Institute of Health, Belhesda, Maryland.                "Rosai-Dorfinan disease "


REFERENC&S:
Foucar E, eta!. Sinus Histioc)10S1S \\ith Massive l,.ymphadcnopath)· !Rosai-Dorlinon Disease). Review of the Entity Scmin Diag
   Ptuho/ 1990; 7(1).17-73.
Montgomery EA. ct ol. R05oi·Oorfmon Disease of Soft Tissue. AmJ Surg Pothol 1992. 16(2):122·129
Bcrsamaschi G. Ct ol. Evidence for a Po1yc1 Noturc of the Cc!11nfiltratc in Sinus ~hstiocytosis with Massive L)mph•denopathy
                                           ono1
   (Rosai.Dorrmnn Disease). Br .I Naematal 1995; ')1 (2):415·4 18.
Vcinol JP, ct at Son Tissue Rosoi Dorfmno Disease Mimicking fnOanunotocy P.seudotumor. A Dia0     onostic Pitfall. PIIIM/ 1998:
   30(1).14-16
Case No. 2, Accession No. 28148                                                                   March 2000

Bakerslield - Nwroendocrine carcinoma
Riversids - Malignant carcinoid
Mountain Yiew lEI Camino Hospital) • Small ccll neuroendocrine carcinoma
Orange IUC! Medical Center l!.esidemsl · Small cell carcinoma
Havward - Basa!oid carcinoma (I}; Small cell carcinoma (2}
Ventura (Unjlab) - Carcinoid (2)
Santa Rosa - Neuroendocrine carcinoma (2); Small cell carcinoma (neuroendocrine) (I}
Santa Barbara (Co!IJ!SC Hospi(QI) - Neuroendocrine carcinoma
Momerex (Community Hospital of Monterev Penin~ulal • Small cell carcinoma
San PieJ?.Q !Nayql Mcdi.c;ll Cell!ID - Net•roendocrine carcinoma
~ - Cat·cinoma with neuroendocrine features (6)
Michigan {Dearborn) - Invasive carcinoma with neuroendocrine and squamous features bigb grade
Texas O'exas Tech Medical Health Center\ - Poorly diflerentiated carcinoma with neuroendocrine difrcremintion
Nebraska {Crejghton University) - Poorly differentiated carcinoma with neuroendocrine features
Florida (Monroe Regional Medical Center) - Neuroendocrine carcinoma
Mro!lan<lllJ.niversilY ofMruyland} - Poorly differentiated neuroendocrine carcinoma
Maryland IWgodbinel - Small cell carcinoma (I}; Neuroendocrine carcinoma (I)
Maryland (National Naval Medical Center) - Small cell neuroendocrine tumor
~ JemY.LOveriQOI< !iQspital} - Cloarogenic carcinoma (I): Small cell neuroendocrine carcinoma !2)
 Pennsylvania ll.ehjgh Valley Hospital) · Small cell neuroendocrine carcinoma
                                    ial
 Pennsylvania (Conemaugh Memor Hospital) - Basaloid carcinoma with neuroendocrine dificrcmiation
New York (Mutl.l<illll!'JLMedical Center) . Small cellneuroendocrmc carcinoma
 New York (Long Island Jewish Medical Center) - Neuroendocrine carcinoma
 Cmnecticu~ (l,lnix.cail~5l.llJl!!.Sl)cYL Heahh Center) • Small cell carcinoma of colon
 Canada (foorbjl!s Ho~pillill - Small cell carcinoma (neuroendocrine carcinoma)
 Japan CShimndn Cjw} - Neuroendocrine carcinoma
 Australia (Sydney) - Neuroendocrine carcinoma (3), Small cell carcinoma ( I)


DIAGNOSIS:
     Smull Cell Carcinoma (Neuroendocrine Carci nom•), rectum
            T-68000,M-80413


 REFERENCES:
 Gould VE.ct ol. Neuroendocrine Carcinoma of tho Colon. Uhl'>struelur>t and Btocl>      enucalEvidcncc of their Secretory Function
     AIO!.I Smog l'ntholt978; 2(1):3t -38.
 Wick MR, or al Smoll Cell Neuroendocrine Carcinoma of the Colon ond Rcc.tum. Ctinicol, Histologicnl. ~nd Ultrnstruclurol
     Comparison with CtoncogenicCorcinomo. Studyof lnmllmohisiochcmicnt Comparison. Hum l'orho/ 1987: 1             8(t):9-2 1.
 Mills SE, ct ol. Small Cell Undiffcre.utiatcd Carciuomo of ~1c Colon. A Ctinicoparhotogieal Study of Five Cases and Their
     Association wi~t Colonic Adenomas. Am .I Surg Pathul 1983; 7(7):643-65 I.
 Sorsr.cld P, cL nl Smoll Cell Undiffcre.nLiatcd (''NC<Jrocndocrlnc") Cnreinon1o of the Colon. Hmopmhol t990: 16(4):357-363.
 Saclllfidcs TJ, ct ol Neuroendocrine Cancers of the Colon ond Rectum Results of a Te11-Year E,,pcricncc. Db Colon Rccwm t994:
    37(7) 635·642.
 Gaffey, MJ. ct at Neurnendocrine Carcinoma of the Colon nnd Rectum. A CtiJUropnthologic:. Ultrnsti\Jctural. and
    lmmunohtstochenucal Studyof24 Cases. AmJ Surg Purh<>/1990. 14(tl) 1010-1023.
                                                                                                 Marth lllllll

Bakersfield • inflammatory fibroid polyp
Riversjde • lnfiammatory fibroid polyp
Moumajn View lEI Camino Hospitnll • Inflammatory fibroid polyp
Qranw=. CUCI Medical Center Rcsjdcms) • Inflammatory myolibroblnstic tumor (inflammatory pseudotumor) (4);
    Liposarcoma. myxoid (4)
 Hayward • lnflammato.ry fibroid polyp (3)
 Vemyrn IUnjlab) • Myxoid liposarcoma (2)
Sama Rosa · Gastrointestinal stromal tumor {3)
Santa Barbara CCottage Hospital\ • Inflammatory fibroid polyp
Monterey (Communi tv Hospital of Monterey Peninsula) • Stromal tumor
S;ln Diego CNaval Medical Center) • Inflammatory fibroid polyp
Long Beach • Inflammatory fibroid polyp {Va.flek) {6)
Mjchi~ • Inflammatory fibroid polyp
~ trcxas Tech Medical Health Cemcr) - Gl stromal rumor
Nebmskn <Creighton University) • lnO   ammatOJy pseudotumor
floddn !Monroe Regional Medical Cemec) • Inflammatory pseudotumor
Marvlnnd !University of Maryland\ • Inflammatory fibroid polyp
Maryland CWoos!bine) • Inflammatory pseudotumor {2)
\<fnryland CNatjona! Naval Medical Center) • Inflammatory fibroid polyp (9)
New Jersey (Overlook Rosl!iW) • Inflammatory fibroid polyp {3)
PennsylygniaiLehigh Vallev Hos!!iW) • Stromal tumor with myxoid changes
Peousvlyooia (Conemaugh M~roorU!ltJQ.ipJtl!n · lnflanunatory fibroid polyp
New York (Montefiore Medjc;!l Center) • GIST vs. intlammatory pseudotumor
New Yotk l Long Island Jewish Medjcul Center> • Jnllammatory fibroid polyp {inflammatory pseudotumor)
Connec)jcyt (llniversitv ofConnecticul Health Center) · Gastrointestinal stromal tumor of low malignant potential
Canada (foothills Hospital) • Inflammatory fibroid polyp
Jaoan (Shimada City) • Inflammatory fibroid polyp
Australia (Svdncy) - lnfiammatory fibroid polyp


DIAGNOSIS:
     lnOnmmntory Fibroid Polyp, S mall Intestine
           T-64000, M·7683()


REFERENCES.
Suster S, etal. lnflanunatory Fibrord Polyp of the SmaU Intestine. Ullra$1tuctural and Immunohistochemical Observations
    Ultrastn•ct Potho/1990: 14(2):109-119
M)1nt MA, t1 a1 Inflammatory Pseudolumor of the Ileum. A Report of a Multi focal. Tronsmurol Lesion \\ith Regrona1 L~mpb
    Node lnvoh·emcnt. Arch Patlro/ LabMtd 199<1: 118(11):1138-1142
Trillo, 1\A, t1 al The Histogenesis oflnOammntory Ftbroid Polyps of the Gastrointestinal Tract. Hmoparho/1991 ; 19(5) 431436.
TadoS, ct al Endoscopic Removal of lnOammntory Fibroid Polyps of the Stomoch. Am .I Grurroenttrol 1991, 86(9): 1247-1250.
Shih LN, ct nl ~>Onmmatory Fibrord Polyp of the Jejunum Causing Intussusception Am J Gasuoenrero/ I997, 92( f): 162. I6~.
Wille P, ct al. Fibroid Polyps ol' lniestinnf Tract nrc /nllnmmatory·Rcoctivc Prolilerotions of CD34-Positive Pcnvnseulor Cells.
    N/.woplllhol 1998; 32(6):498-502.
Case No. 4, Accession No. 28153                                                                    Marclt2000

~J:lili.!lli[ -Serous cystadenoma
Riverside - Lymphangioma
Mountain View CEI Camino Hospital) - Benign multicysric mesothelioma
Orange (HCI Medical Cegter Re.~ - Lymphangioma
Hayward - Cystic lymphangioma (3)
.Ymtllra <Unilab) - Lymphangiomatosis (2)
S!'nt~ Ros;t - Lymphangioma (3)
Santa Barbara (Cottage Hospital) - Benign cystic mesothelioma
Monterey (Community Hospital of Monterey Peninsula) - Lymphangioma
San Diego (Naval M!:liic;J,I <&nter) - Benign multilocular peritoneal inclusion cyst
Lone Beach - Benign multicystic mesothelioma (6)
Mil<blltan CDearbornl - Mtilticystic mesothelioma
Texas (Texas Te~h Medical fl'<Jltb Cent~rl - Cysfic mesothelioma/lymphangioma
Nebraska (Creighton University) - Multilocular peritoneal inclusion cysts
Florida !Monroe Rel<ional Medical Center\ - Lymphangioma
Mar:ylang <llniyersi!.Y of Mqr:y!ao\!1 - Multicystic benign mesothelioma (multiple peritoneal ittclusion cyst)
Maryland CWoodbinel - Multicyst1c mesothelioma (2)
~~ma.lld <National Naval Medical Center) - Mulricystic peritoneal mesothelioma (8); Cystic lymphangioma (2)
New Jmey (Overlook H       rul.li.l.ltll - Benign multicystic mesothelioma (3)
Pennsylvania (Lehigh Valley Flo spital\ - Benign multicystic mesothelioma
Cenosylvania (Conemaugh Memotial Hospital\ - Muhicystic benign mesothelioma
New York CMqntefiore MWigal C~nter) - Cystic mesothelioma
New York (Long Island Jewish Medical Center! - Multicystic mesothelioma
~tjglt Cllniversilv ofConnecticm Health Center) - Multicystic mesothelioma
Canada Cfoo!hms Hosojtal) - LympharJgioma
Japan (Shimada Cit.;:} - Benign rnulticystic mesothelioma
Australia (Sydney} - Multicystic mesothelioma


DIAGNOSIS:
          Benign Multicystic Mesotbclioma
                    T -67000, M-90503


REFEREN~:
Moore JH.Jr., ct al. Benign Cystic Mesothelioma. Cancer 1980; 45(9):2395-2399,
McMemcycr R, et nl Multicystic Peritoneal Mesothelioma. A Repoi'L witJ1 Electron Microscopy of a Case Mimicking Intra·
    Abdominal Cystic Hygroma (Lyrnphan,~;ioma). Ctmcer 1979: 44(2):692-698.
Weiss SW~ ct al. Muhicystic l\•1csothcliomn. An Analysis of Pathologic Findings and Biologic Behavior in 37 Cases, Am ../ Surg
    l'otho/ 1988; 12(10):737-746.
Wu YJ, ct at. The Me~od~elial Keratins. A New Family of Cytoskelelal Protei us ldentiGed in Cultured Mesothelial Cell, .and Non-
    Keratinizing Epithelia. Ci>ll 1982; 31(3 Pt 2):693-703.
ZotaJis G, et al. Lciom~·omalosis-Pcritoncalis Disscminata, Endometriosis, and MuJticysl,ic Mesotllelioma. An Unusual Association.
    lnt.!Gynecol f>mho/ 1  998: 17(2):178-1 82.
Cas ~   No. S, Accession ~o. 28680                                                             Mar~h   2000

Bakersfield • Mucinous cystadenoma
Riverside • Mucocele
Mounlain View lEI Gamino Hospital) • Mucinous cystadenoma with mucocele
Qmn!llLCUC! Medical Center Residems\ • Acute appendicitis with perforation and granulomatous inflammation
~~.n! . Mucocele, mucosal hypet·p!asia type (non-neoplastic) (3)
Venturn ll!oj!ab) - Mucocele (2)
S•nta Rosa • Mucinous cystadenoma by history (3)
Santa Barbara (Cottage Hospital) • Mucinous hyperplasia
Montercv CComrnunily Hospital of Monterey Peninsula) · Mucinous cystadenoma
San Diego CNaval Medical Cemer) • Mucinous cyst.adenoma (8); Mucinous hyperplasia (S)
Long Deacll . Circumferential villous adenoma (6)
Michigan <Dearborn\ • Mucosal hyperplasia           •
Texas !Texas Tech Medical Health Center) • Pseudomyxoma peritonei
Nebca§kil..(Cteighton U!liversity) • Mucinous cystadenoma of appendix (mucocele)
Florjdn (Monroe Regional Medlen! Center\ - Mucocele
Maryland CUnjyersirv ofMaQ'Iand) · Mucosal hyperplastic of the appendix
Mat¥land (Woodbine) • Cystadenoma (2)
Marvland {National Naval Medical (~DJJ:[} - Mucinous cystadenoma with hvperplastic features
'-ew Jersa(Qverlook Hoswtll!) • Mucocele with hyperplastic mucosa (I): Mucocele "ith adenomatous
   transformation (2)
~enn•ylxnnia (lehigh Valley Hospital} - Mucinous cystadenoma of appendix (I)
~Jl!on{o (Conemaugh Memo pal I !osojta!l • Arteriovenous malfornuuion
New YoJk CMonteliore rywlical Ccmcr\ • Diverticulum associated with low grade mucinous cystadenoma
New '(Qrk.(klmg Island Jewish•.MJ:!Iieal Center) - Mucinous cystadenoma with rupture
Conncc)icul (Universit" ofConnectjcuL I lcal!h Centru • Mucinous cystadenoma of appendix, borderline malignant
    potential
Canada (foothills Hosojtall • Hyperplastic polyp, venniform appendix
Japan <Shimada City) - Schistosomiasis
Australia CS~ - Tubulovillous adenoma associated with a mucocele


DIAGNOSIS:
     Mucinous Tumor of Unceo1nin Mlll ignant .Poleotial, Appendix
            T-66000, M-800 I I


REFERENCES:
Htgo E. C1 ol. Mucosal Hype<plasi"'Muctnous Cystadenoma. Mucinous Cystadenocarcinoma ofthcAppcndi' A Rc-F.\alu>tion of
    AppcndoecaJ··Mucoccle'' Can«!r 1973: 32(6):1525-1541.
Htllhold Dt.. ct ol Appcndiecallnt~JSS<~SCCpiOon liS a Marufestation of Muclllous Crstadc1101ru1 of the Append" An lntcrcstmg
    Clinocat Enuty. Am Surg 1997; 63(5):390-391
Guemcri C. ot al Mucinous tumors of the Vermiform Appcndi.x and 0\'ory. and Psoudomyxonoa Peritonei. Histo~oenctic
                                   .
    lnoplkotoons of Cytokcrotin 7 E,prcssion. Hllm l'athll/1991: 28(9): 1039-1045.
                                                                            m
Shirnit.u T, ct nl. Mucinous Cystadenoma of Lhc Appendix with Raised Scn.1 Cnrcinocmbryonic Antigen Conccnlrotton Clinical
    and PnUtologicol Footures. J Clin Pnthol 1991; 50(7):613·614.
                              o
Smith JW. ot ol. PscudOID)'l<O\lo Peritoncl ol' Appcndtceal Origin. The Mcmori11l Sloan-Kettering Cancer Center Experience.
    Cnn<,.,. t992: 70(2):396-40 L
Costo M, ct ol C}10iogyofPseudonoyxoono Pcritone.i. RcportofTwo Cases Arising froon Appendiceal Cystodcnomo J)ragn
    Cytopotlool 1990; 6{3):20 1-203.
Stevens KJ. et ol Psoodomyxoma E.xtnlpcritond A Lethal Complication of Mueonous Adcnoearciooma of ~1e Appendix. Am J
    GnJrroen,.ro/1991; 92(10): 1920-1922
Case.No. 6, Accession .No. 17251                                                                  March 2000

Bakersfieli! - Bile duct hamartoma
Riverside - Angioma
Mountain View lEI Camino_Hospit!!!} • Infantile hemangioendothelioma
Orange !UCI Medical Center Residents) - lnfaniile hemangioendothc.lioma
Hayward - Infantile hemangioendothelioma (3)
Ventttrn (!}.D.iJJ\l!l - lnlimtile hemangioendothelioma (2)
Santa Rosa - Infantile hemangioendothelioma (3)
Santa Barbara (Cottage Hospitall - Infantile hemangioendothelioma
Monterey (Community Hospital of Monterey Peninsula) - Biliary atresia
San Diego Q!!aval Medical Center) - (nfantile hemangioendothelioma
Long Beach - Infantile hemangioendothelioma (6)
W!i&~IJ !Dearborn) - Cirrhosis with massive ductal proliferation (cystic fibrosis ?, infantile obstructive
       cholangiography ?)
Texas Cfexas Tech Medical Health Center) - Hamartoma
Nebraska (Creighton University) - Infantile hemangioendothelioma
FIQ CMmu.~gionjjl Medical Center) - Extrahepatic biliary atresia
      rida
Maryland !University of Mary laud) - Infantile hemangioendothelioma
Maaland (Woodbine) - lnf.1ntile hemangioendothelioma (2)
M....ar,y.l.!!!liHNa!jooa) J::!ava! Megical Cen.tW - Infantile hemangioendothelioma
New Jersey (Overlook Hospital) - Infantile hemangioendothelioma (3)
Pennsylvania (Lehigh Valley Hospl!!J.U - Extrahepatic biliary mesia
Pennsylvania (CQnemaugh Memorial Hosnitall - Bile duct hamartoma
New York (Montefiore Medical Center) - Multiple hemangioendothelioma of the liver
~ew York (j.gng [slaod Jewish Medical Center) - Infantile hemangioendothelioma
~lis.Y.L(llnjyersity of Conne~ticut He~lth Cen(erl - lnfantile hemangioendothelioma
Canada (Foothills Hospital) - Infantile hemangioendothelioma
Japan (Shimada City) - lnfan!ile hemangioendothelioma
Australia !Sydney\ - lnfuntile hemangioendothelioma


DIAGNOSIS:
          lllfantile Hemangioendothelioma, Liver
                    T-56000, M-91310


REFERENCES:
Dochmnn AH, ct al.. Infantile Hemangioendothelioma of the Lil'er. A Radiographic-l'olhologic.·Cfinical Correlation. AJR 1983:
    140(6): 1091·1096.
Denn PJ. cl al MaJignaot Rcmangiocndothclioma of the Liver in a Young Woman~ ReJa~iouship to Ora) C'.onlrnccptivc-Us-c. Am J
    Surg Patho/ 1985:9( I0):695· 704.
Amonknr P, et nl Infantile Hema11gioendod1elioma of the Liver Med l'edlmrOncol 1999; 32(5):392-394.
Wollcring MC, ct nl. Hepatic Hemangioendothelioma of Infancy. Treatment with Interferon Alpha. ./ Pedlmr Ga.srroenteTOI Nmr
    I997 24(3):348·351.
Selby OM, et al. Infantile Hemangioendothelioma of the Lil'er. Hepato/ 1994; 20(1 PL 1):39·45.
Case No. 7, Accession No. 26302                                                                     Marth 2000

Bakersfield • Hemangioma
Riverside • Hemangioma
Mountain View CEI Camino HospitaD - Cavernous hemangioma
Orange CUCI Medical Center RC§idents) • Cavernous hemangioma
Hayward • Intravenous leiomyomatosis, metastatic (2); Hemangioma ( I)
Ventura CUnilab) • Hemangioma (2)
Santa Rosa • Hemangioma ( 1); Hemangioma, rule out "low grade" angiosarcoma ( 1); Low grade hemangiosarcoma,
     rule out hemangioma (I)
Santa Barbara CCottuge Hospil.l!!} • Cavernous hemangioma
Monterey (Community Hospital of MontereY Peninsula\ • Hemangioma
San Diego INayaJ Medjcal Cemer) - Cavemous hemangioma
Long Beach • Cavemous hemangioma (6)
Michigan CDearbom) · Hemangioma
Jexa• !TexM Tocb Medjcal Health Center) Papillary mesothelioma
Nebraska {Creighton University) • Cavernous hemangioma
flllljda {Monroe Reuional Medical Center) • Cavernous hemangioma
Marvland (Universily of Marvland) ·Cavernous hemangioma
Matyland (WoQdhjne) • Cavernous hemangioma (2)
Marvland ® tiona! Nayal Medical Center) · Hemangioma
New Jersey {Qyer!ook HospJW) • Hemangioma (3)
Pennsylvania ll.chigb Ylllley Ho~pital) • Hemangioma
Pennsylvania IConQ  rnaugh Memorial Hospital\ - Hemangiomatosis
J:!ew York !MoOJefiore Medjcal Center) • Cavcmous hemangioma with (no! legible)?
New York (Lon• Island Jewish Medical Center) · Cavemous hemang.ioma
Conneclicyt (UniyersiJy of Connecticut Health Center) • Sinusoidal papillary hemangioma
Canada (l'oothjl!s Hos!lW!.U • Hemangioma
Japan (Shimada Cjty) • Lymphangioma
Australia (Sydney> • Cavernous hemangioma


DIAGNOSIS:
     Cave.rnou.s Hemangioma, Liver
                    T-56000, M-91210


REFERENCES:
flobbs KE. ec nl, Hepntie Hcman~>iomas. Worl<l J Surg 1990; 14:468-4 71.
                                                                        diologic Investigations, and Treatmcnl of 20 PaclenlS. Ctmccr
Stonley P, el al , lnf11111ile Hepatic Hemangioma. Clinical Features, R11
    1989; li4(4):936-9·19.
1\vaC.'Uki S, cl ul. Excisional Therapy of Benign Hepatic Lc.<ions Sur11 <iynut.'Q/ Obs~et I99Q; 171(3):240-246.
Johnson CM, ct ol. Computed Tomography and AngiographyorCovcmous Hcntanb>iomas of the Liver. !1adtology 1981;
     138(1): 115·121
Gibney RO, cc al Sono&fllj>hically Dcteeted Hepauc Hemangiomos. Absence ofChMge Over Time. A./11 1987; 14 9(~):953·957
YomilJllOIO T. et al SpontanCOU$ Ruptureofbemangiomo of the Lh·cr. Trealmc:nt "ith Transcolhetu HepaucAncnal Emboli>Ation.
    AmJ Gastrotnttrol 1991; 86(11):1645-1649.
Ko1v YT, et al. C)1ologte Diagnosis of Cavernous HemMgi<lma of the Li1-cr ll'l!h Fmc-Needle Biopsy. Diagn C)•opmhol 199 J;
    7(6):628-630.
O.se No. 8, Acces.•ion No. 28489                                                                   March 2000

Bakersfield - Fibrolamellar variant hepata<:ellular carcinoma
Rjverside - liepatoeeUular carcinoma, sclerosing type
Mountain View CEI Camino Host>ilall - Hepatocellular carcinoma
Orange CUC! Medjcal Center Residents) - Hepatocellular carcinoma (6); Acinar cell carcinoma (2)
Hayward - Fibrolamellar carcinoma (3)
Ventura CUnilab) - Acinar cell carcinoma (2)
Santa Rosa - Acinar cell carcinoma (3)
Santa Barbara (Couage Hospital} - Malignant neoplasm, lavor carcinoma, site undetermined
Momecey !Community Hospital ofMonterey Peninsula) - Pheochromocytoma
San Dic1,10 (Nayal Megical Center) - Hepatocellular carcinoma (8); Adenocarcinoma (7)
Long l3epch - Fibrolamcllar hepatocellular carcinoma (6)
~!m CDcub.ru:.o) - Acinar cell carcinoma. pancreas.
Tc.xas (Tc~as Tech Medical Health Cent~rl - Neuroendocrine tumor
 'ebraska CCrejghton Universitvl - Pheochromocytoma/acinar cell carcinoma
Florida (Monroe Regional Medical Center) - Hepatoid adenocarcinoma
Maryland CUpjyersitl'-(lfMarylaod) -Hepatocellular carcinoma (5), Acinar cell carcinoma (5)
Marvtand !Wooc!bine) - Sclerosing hepatocellular carcinoma (2)
M.!!!yland (N~tional "'aval Medical Cemer} - llcp;1tocellular carcinoma. lihrolamellar variant
New Jersey (Qy_erlook HQSJli1il!l - Hepatocellular carcinoma (2); Unclassified earcinnmn (I)
~QnsYI¥anja CJ,migh Valley Hospital\ - Endocrine carcinoma
Penn,y!ynnia (Cpncmaugh Memorial Hospital) - Acinar cell carcinoma, pancreas/Malignam melanoma, mctastat ic
New York ili!<l.U.ll:llr.>JJ\ Medical Center) - Adrenal cortical adc.non1o vs. adrcnol cortical carcinoma vs hepmoid
   carcinoma
              ..
New York o gnsl@lud Jewish Medical Center) - Hepatocellular carcinoma favor fibrolamcllat· typc but lacking
   oncocytic leatures
Connecticut !llnjyersjty of Connecticut He-dlth Center) • f'ibrolarncllar hepatocellular cilrcinoma
Canada !Foothills Hosl!ill!!) • Acinar cell carcinoma
W!M..<.S.Iliroada City) - Poorly differentiated carcinoma
Australia CSydpeyl - Hepatocellular carcinoma (3). Fibrolamellar heparocellular carcinoma (I)


DJACNOSIS:
     Fibrolamellar Cardooma, Liver
                     T-56000.~1-80103



REFERENCES·
Crais J, c1nl. f'ibrolnrncllnr Carcinoma of the Lil'cr. ATumor of AdotesCCI)IS~nd Yow>s Adults will1 Distinctive Climcot>
                                                                                                                        otholosic
    Features. ('mwer I~RO ; 46(2):372-379.
Bcrm~1n M, ct nl.                                                                             ccn
                    Fibrolnmclln.r Carcinoma of Lhe Liver J\n Immunohistochemical Study ofNineL Cases nnd n Rovicwoi' Lhe
    Literature. 1/um l'mhn/ 19K8; 19(7):784-794.
Torii A. et nl. Tumor Localization as o Prognostic Factor in Hcpotocellulnr Carcinoma. Heparogostroenterology 1994; 4 t( I): 16· 19.
Epstein BE, c;.t nl. Mctostntic Nonrcscclablc Fibrolruucllar Hepatoma Pro,rnostic Features 3nd Natural H1story. Am J Clln Um·oJ
    1999: 22(1 ):22-28.
Davison FD. et nl. HBV-DNA Sequences in Tumor and NonrumorT•ssuo in o Patient "ilh the Fibrolamcllar Voriont of
    HepatoccUul3r Carcinoma. Heparo/ 1990; 12(4 Pt I) 676-679.
Oa\'onp<Jrl RO C)1ologjc Diagnosis of Fibrolamellar Carcinoma of lhe ln cr by Fine-Needle AspuaiJOU. IJ/ogn C') topatho/ 1990;
    6(4)27S-279
Okano A. et al Fibrolamcllar Carcinoma of lhe Li\·c r with • "'"turc of Ordmary Hepatocellular Olrc1noma A Case Rcp<Jrt. Am J
    Gcwrocntt•ro/1998; 93(7):1144-11 4S.
Cl5t No. 9, Accession No. 28553                                                                      Marth 2000

Bakersfield - Clear cell carcinoma
Riverside - Hepatoma
Mountain View CEI Camino Hos!lilAI.l - Hepatocellular carcinoma, clear cell type
Orange CUC! Medical Center Residents) - Hepatocellular carcinoma
~s! - focal nodular hyperplasia, liver ( I): Metasta~ic renal cell carcinoma (2)
Yentyra (Unilab) - Hepatocellular adenoma (2)
Santa RoM - Adenoma vs. focal nodular hyperplasia (3)
Santa Barbara (Cottage Hospilal) - Hepatocellular carcinoma. clear cell pattem
Momerey /Community Hosojtal ofMonu:rey Peninsula) - Hepatoma -clear cell
San Dje11o CNaval Medical Center) - Metastatic renal cell carcinoma
Long Deneb - Hepatoma, grade I (6)
Mjg]]!Uin.IDearboml - Hepatocellular carcinoma, well-diflerentiated
Texas < Texas Tech Medical Health Center) • Hepatocellular carcinoma, clear cell type
Nebruska.ICreiwhton Univecsit'tl - Hepatocellular carcinoma, clear cell variant
Florida (Monroe Regional Medical Cemcrl - Hepatocellular adenoma
Macyland (Uojyersity ofMarvland) - MultiJ>Ie hepatocellular adenomatosis vs. well-differentiated hepatocellular
    carcinoma
t.!Aorland IWoos!bine) - Clear cell hepatocellular carcinoDl8 (2)
Macyland INattonal Naval Medical Center) - Clear cell hepatocellular carcinoma (9); Metastatic renal cell carcinoma
   (I)
~.um         (Overlook Hospital\ - Hepatocellular carcinoma (3)
Pennsylvania /Lehjgh Valley Host:tit~D - llepatocel!ular carcinoma
Pcnnsy!vonju <Conemaugh Memorial ~.jostlitlll) - Metastatic adrenal corticol carcinomaiRenal cell adcnocarcin<  >ma,
    mctosuuic
New York /Montefiore Medical Cem~C) • Clear cell hepatoma vs metastatic renal cell carcinoma
New VQrk /Long Island Jewish Medical Center) - Hepatocellular carcinoma of clear ceO type
         i
Connes:t.cut CUnjyersitv ofConaecticuJ..l:iealth Center) - Well-differentiated hepatocellular carcinoma/Liver cell
    adenoDl8 must be considered
Canada Cfoothills Hospital) - Clear cell hcpatoceUular carcinoma
Iapan CShiroada City! - Adrenal cortical carcinoma or clear cell hepatocellular carcinoma
r\uslmlill ISydne'tl - Hepatocellular carcinoma (2); Metastatic renal cell carcinoma (2)


DIAGNOSIS:
     Clear CeUVariant of Hepntocellular Carcinoma, Liver
                   T-56{H}O, M-81700


REFERENCES:
Cnl\1 fOfd J. Ct ol. Pathologic .<\ssessment ofLorcr Cell Dysplasia and Bcnisnu,cr
                                                                                 Tumo<s Di(fcn:nuatlon from mallgiUIIlt
    Tumors S•Mitt Diog Patho/1990, 7(2): II S-128
Smgb HK, ct at. Fine-Needle Aspiration Cytomorphology of Cleor-Cell Hepaooc:ellulllf Carcinoma. Diagn Cytopotllo/1997;
    17(4):306-3 10.
Adamek H1~. ct nl. Primary Clear Cell CnrcinomR of Noncirrhotic Li\'cr. Immunoltis1oc11en:tical Discrimination of Hept'llocellular
    ond Cbolnngiocellular Origin. Dill /Jis Sci 1 9?8; 43(1);33-38.
Guptn RK, et al. Aspimtion CytodiagnoSIS of Clear Cell hcpalooellular Corcinoma in an Elder!)' Woman. A Case Report Acta
    (}tO! 1994; 38(3}:467-469
McDermott WV. et al. Clear Cell Carcinomn oft he L•vcr with SponlonOOUJ Rel:JtSsion of Metastases. J Surg Oncol 1994,
    57(3)·206-209.
DonatEE. ct al C)1odoag110SIS ofCieor Ccll HcpatocellularCarclllOOla. A Case Report. Aeta C)•o/1991 ; 35(6);671-675.
Case No. 10, Accession No. 26335                                                                     March iOOO

Bakersfield - Mucinous cystadenoma
Riw~ - Mucinous cystadenoma
Mow>tain View CEI Camino Hospital) - Mucinous cystadenoma
Orange fUCI Medical Center Residents) - Mucinous cystic neoplasm with no stromal invasion
lfll~ - Low grade mucinous carcinoma (3)
Ventura CUoilab) · Mucinous cystic tumor, adenoma (2)
Santa Rosa - Mucinous cystadenoma (1); Cystadenoma (2)
Santa Barbqm {Cpttage H'osoitall - Mucinous cystic neoplasm, borderline
Monterey (Community Hospital of Monterey Peninsula) - Mucinous cyst.adenoma
San Diego (Naval Medical Center) • Mucinous cystic neoplasm (mucinous cystadenoma) (9); Borderline mucinous
    cystic rumor (4)
J,ong Beach • Mucinous cystadenoma (6)
Michigan (Dearbom\ · Mucinous cystic neoplasm
Texas ITexas Tech Medical Health Center) • Mucinous cystic neoplasm
~ska (Creighton Universit)'l · Mucinous neoplasm of pancreas
Florida (Monroe Regional Medical CenJ&r} • Mucinous cystadenoma
Marvland CUnjversity ofMa..IJ1arull ·Mucinous cystic tumor
M.iln:J,md (Woodbine) • i'vfucinous cystic tumor, borderline (2)
Maryland (National Naval Medical Center) - Mucinous cystic neoplasm
New Jersey (Overlook Hospital) • Mucinous cystadenoma (3)
fe!'n~~~~unil\.{!..ehigh Vallev Hospital) · Mucinous cystadenoma, borderline
Pennsylvania (Conemaugh Memorial Ho~P.ilJ!D. · Mucinous cystadenoma, microcystic L}'Pe
New York IMontefiore Medical Center) • Mucinous cystadenoma
New York (Long Island Jewish Medical Center) · Mucinous cystadenoma
Connecticut (University of Connecticut Health Ce!UW • Mucinous cystic neoplasm, low malignant potential with
    ovarian srroma
~bills HQ~pjtal)                • Mucinous cystic tumor, benign
Japan <Shimada City) · Mucinous cystoma
Australia (Sydney') - Mucinous cystic tumor with ovarian·l}'pe stroma


DIAGNOSIS: '
     Mucinous Cystadenoma, Pancreas
             T-59000, M-84700


~~:                        .
Bergmann LS, eta!. CystadCllomas of !he ~nncre.'lS. The American Surget!ll 1992;58:57-71.
Albores·Saavcdra J; ct al. 'Rc.commendnli01 for the Rei)Orting of Pancreatic Specimens <;ontaini1tg MolignMl ·1
                                          \S                                                                  \amors, The
   AssQCiation of Directors of Anatomic and Surgical Pathology. Am .! Clin !'athol I 9?9; I J 1(3):304-307.
Albores-Saavcdra J~ ct al. Mucinous Cystodcuncnrcinomo of the Pancr   eas. Morphologic and Immunocytochemical Obscrvntions.
   Am.J Surg Patho/ 1987; I I(1 ): ll-20.
Obara T, et -al. MucinwProducing Tumor of the Pancreas. Natural History and Serial PancrenlOgram Chongcs. Am J Gas1roenrerol
    t993: 88(4):564-569.
Cyst Fluid Cytologic Analysis in the Oit'ferentiat Diagnosis of Pancreatic Cysuc Lestons, Am .J Clin !'athol t994: I 0 1(4):483-487.
Shyr VM, ct al. Mucin-Producing Ncoplasmsoflhe Pancreas. Intraductal Papillary and Mucinous Cystic Neoplasms. Ann Surg
    t996: 223(2): 14)-146.
\V'oulc.rs K._ CL al. A Pnncreadc Mucinous Cystadenoma in a Man with Mesenchymal Strom~. E~pressins Oesltogcn and
    Progesterone Receptors. Vichows Arch I998; 432(2): I87· I89.
Fukumoto T, et al. Clinicopathological Study on Cystadenocarcinoma of the Pancreas. Gastroemeroi .Jpn t987; 22( t):63-73.

				
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