CALIFORNIA TUMOR TISSUE REGISTRY

"SOFT TISSUE PATHOLOGY"

Study Cases, Subscription A

APRIL1999

California T umor Tissue Registry c/o: Department of Pathology and li urn an Anatomy

Loma Linda University Scbool of Medicine Jl021 Campus Avenue, AH 335 Loma Linda, California 9'2350

(909) 824-4 788 FAX: (909) 558-0188

E-mail: cttr@linkline.com

'

Target audience: Practicing pathologists and p,athology residents.

Goal: To acquaint the participant with the hiSiologic features of a variety of benign and

malignant neoplasms and tumor-like conditions.

Objectives: The participant will be able to recognize morphologic features of a variety of benign

and malignant neoplasms and tumor-like conditions and relate those processes to peninent references in the medical literature.

Educational mtthods and media: Review of represenlative 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: Lorna Linda University School of Medicine designates this continuing medical

education activity for up to 2 bours of Category I of the Physician's Recognition Award of the American Medical Association.

CME credit is offered for the subscription year only.

Accredi!Jition: Lorna Linda University School of Medicine is accredited by the Accreditation

Council for Continuing Medical Education (ACCME) to sponsor continuing medical education for physicians.

CONTRIBUTOR: Richard L. Johnson, M .D. CASE NO.1 - APRIL 1999 Pasadena, CA

TISSUE FROM: Right back ACCESSION #27925

CLINICAL ABSTRACT: This 40-year-old Caucasian male had a lump removed from his lower back. The lump returned

and grew larger and ftrm to touch. MRI revealed a relatively well-circumscribed, solid mass, posterior to the spine.

GROSS PATHOLOGY: The sped men consisted of a 72 gram, 8.5 x 5.0 x 3.0 em gray-pink soft tissue mass. The cut

surface was fibrous and gray to light tan.

CONTRffiUTOR: Donald Rankin, M.D. CASE NO.2 - APRIL 1999 Fontana, CA

TlSSUE FROM: Obturator region ACCESSION #27919

CLINICAL ABSTRACT: During a workup for menometrorrhagia and dysmcnotrrhea, this 44-year-old female was found

to have an obturator mass.

GROSS PATHOLOGY: The specimen consisted ofa 9.0 x 5.5 x 3.5 em yellow-tan, partially gelantinous piece of non­

encapsulated tissue. Sectioning revealed a nodular variable ye llow and white cut surface with no areas ofhemorrbage or grossly apparent necrosis.

SPECIAL STUDIES: CD-34 positive

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CONTRIBUTOR: Philip G. Robinson, M.D. CASE NO.3 - APRIL 1999 Boynton, Beach, FL

T ISSUE FROM: Right shoulder ACCESSION #28414

CLJNICAL ABSTRACT: This 70-year-old male presented with a mass on the right shoulder and left chest. The mass

was excised.

GROSS PATHOLOGY: The 4.2 x 3.5 x 3.2 em specimen consisted of white rubbery tissue. Sectioning revealed a

central mucoid area.

SPECIAL STUDIES: S-1 00 negative Keratin negative Desmin negative

CONTRIBUTOR: Guillermo Acero, M .D. CASE NO.4 - APRIL 1999 Santa Paula, CA

TISSUE FROM: R ight cheek ACCESSION #28J41

CLINICAL ABSTRACT: This 80-year-old Caucasian male gradually developed an ulcerated right cheek lesion, which

was removed.

GROSS PATHOLOGY: The 5.3 x 4.0 x 1.8 em ellipse of skin had a well-demarcated, 3.1 em diameter, ulcerated central

area.

SPECIAL STUDIES: PAS Desmin S-100 Keratin

strongly positive for glycogen weakly. focally positive negative negative

CONTRffiUTOR: Arno Roscher, M.D. CASE NO.5 - APRJL 1999 Granada Hills, CA

T ISSUE FROM: Smull bowel ACCESSION #28297

CLINICAL ABSTRACT: This 51-year-old male had a several year history of steroid and NSAID use with episodes Gl

bleeding and development of an iron deficiency anemia. He collapsed after developing abdominal pain with vomiting and bright red blood per rectum. At the time of surgery, a 12 em firm mass was · found, attached. to the small bowel.

GROSS PATHOLOGY: Attached to the small intestine was a 9.0 x 12.0 em thinly encapsulated yellow-tan mass. The

mass involved the small bowel wall, with a focus of hemorrhage appeared on the mucosal side and extension to the serosa. The cut surfaces showed multiple yellow-tan nodules up to 3.5 em in diameter with foci of necrosis.

SPECiAL STUDIES: Virnentin Muscle specific actin S-100 CD-34 Keratin

diffuse, strongly positive positive negative negative negative

CONTRffiUTOR: Wafa Michael, M.D. CASE NO.6 - APRlL 1999 Fontana , CA

TISSUE FROM : Left inguinal region ACCESSION #27886

CLimCAL ABSTRACT: This 84-year-old male noted a left inguinal bulge for more than a year. It was not reducible and

caused him occasional discomfort but he had had no changes. in bowel habits, abdominal pain or melena. He was taken to surgery for an inguinal hernia repa i:r.

GROSS PATHOLOGY: The 380 gram. 16.0 x 12.0 x 7.0 em specimen consisted of an irregular tan-pink-yellow mass of

fibromembranous and adipose tissue.

CONTRffiUTOR: B. P. Carman, M.D. CASE NO. 7 - APRIL 1999 Upland, CA

TISSUE FROM: Mediastinal tumor ACCESSION #27968

CLINICAL ABSTRACT: This 36-year-old male Caucasian, with a longstanding diagnosis of neurofibromatosis, was

found to have a mediastinal mass. Three years earlier, he had had an 8.0 em thigh mass resected, followed by post operative radiation. At the mediastinal exploration, tumor was found seeding the pericardia I fat, left pleura, diaphragm, anterior pulmonary ligament and pericardium.

GROSS PATHOLOGY: The 8.0 x 4 .0 x 2.0 em specimen consisted of firm homogeneous gray-white to tan tissue

without areas of gross necrosis and hemorrhage.

CONTRIBUTOR: Lorna Linda Pathology Group {drc) CASE NO.8 - APRIL 1999 Lorna Linda, CA

TISSUE FROM: Maxilla and bard palate ACCESSION #27888

CLINICAL ABSTRACT: This 78-year-old Caucasian male had had a lip lesion resected about I year earlier. He now

complained of a tight lip with difficulty in pronouncing some words. He was found to have a rapidly enlarging recurrent cystic mass involving the columella, nose and passing into the nasal cavity. The patient underwent a total rhinectomy, bilateral medial maxi llectomy.

GROSS PATHOLOGY: This 97 gram specimen included nose, nasal septum and hard palate. A 2.0 x 1.0 x 0.6 em

lobulated red mass extended from the nasal septum.

CONTRIBUTOR: Daniel J Luth ringer, M.D. CASE NO.9 - APRIL 1999 Los Angeles, CA

TISSUE FROM: Right chest wall ACCESSION #28338

CLINICAL ABSTRACT: This 26·year-old male presented with a 22.0 em right chest wall mass. The mass was biopsied

and subsequently resected.

GROSS PATHOLOGY: The 30.2 x 12.6 x 12.6 em chest wall resection included of portions of four ribs with attached

skeletal muscle and soft tissue. The medial a~pect of the specimen had a 15.0 x 5.2 x 4.1 em firm white lobulated mass which involved the parietal pleura. The cut surfuce of the tumor was firm, white­yellow, lobulated and friable.

SPECIAL STUDIES: (as evaluated by contributor) LCA Chromogranin I Synaptophysin S-1 00 Protein/ Keratin AEl/3 KP-1 Smooth Muscle Actin I Desmin I Myoglobin Ewing's epitope 013 (CD99) CAM 5.2 NSE Minimal glycogen is identified

Minimal reactivity Minimal reactivity Minimal reactivity Minimal reactivity Mjnimal reactivity Minimal reactivity Minimal reactivity Strong immunoreactivity Perinuclear dot-like distribution Weakly positive in some groups of cells

CONTRIBUTOR: Lorna Linda Pathology Group (mtm) Lorna Linda, CA

CASE NO. 10 - APRIL 1999

TISSUE FROM: Right buttock ACCESSION 1#28367

CLINICAL ABSTRACT: This 64-year-old black male bad a one year history of a mass in the right buttock. There was

no evidence of skeletal involvement on CT scan.

GROSS PATHOLOGY: The 46 gram, 5.7 x 1.2 x 0.5 em. brown-tan ellipse of skin had an attached 5.8 x 5.0 x 4.0 em

ye llow-red, encapsulated, gelatinous cystic mass filled with necrotic and hemorrhagic material.

CALIFORNIA TUMOR TISSUE REGISTRY

SOFTTISSUEPATHOLOGY

Minutes - Subscription A

Aprill999

SUGGESTED READING (General Topics from Recent Literature):

C-Reactive Protein and Cardi.ovascular Risk in Women. Ridker PM, et :al. Circulation 1998;98(73): 1-3. Tumor Markers of Ovarian Center. The Search Goes On. Xu Y, et al. JAMA 1998; 280:719-723. Endooervical Gi¥1dular Atypia. Does a Prenooplastic Lesion of Adenocarcinoma In-Situ Exi.st? Goldstein, NS, Ahmad

E, Hussain M, Hankin, RC, etaL AmJC/in Patho/1998; 110(2):200-209. The Cellular Basis of Metastasis. Ruiz, P and Gunthcrt U. World J Urology 1996; 14{3):141-150. Chan, JKC. Mesenchymal TumO<s o(the Gastrointestinal Tract. A Paradise for Acronyms (STUMP, GIST, Gant, and

Now GIPACT), Implication ofc-kit in Genesis and Yet Another of the Many Emerging Roles of the Interstitial Cell of Cajal in the Pathogenesis of Gastrointestinal Disease. Adv in Anatomic Pat hoi 1999; 6( I): 19-40.

Gastrointestinal Pacemaker Cell Tumor (OIPACT): Gastrointestinal Str.omal Tumors Show Phenotypic Characteristics of the Interstitial Cells ofCajal. Kindblom LG, Remotti H, Aldenborg f', and Meis-Kindblom J. Am J Parho/1998: 152(5):1259-1269.

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California Tumor Tissue Registry c/o: Department of Pathology and Human Anatomy

Lorna Linda University School of Medicine 11021 Campus Avenue, AH 335 Lorna Linda, California 92350

(909) 558-4788 FAX: (909) 558-0188

E-mail: cttr@linkline.com

CASE NO. l , ACCESSION NO. 17925

!NI.ANP <Rhmjde/San Bernardino! • Extra-abdominal fibromatosis (desmoid tumor) (2) ORANGE • Extra-abdominal dcsmoid vALENCIA • Fasciitis, rule out angiosarcoma

APRIL 1999

SA!fiA ROSA . Solital)' fibrws tumor (2): Benign mesenchymal tumor consistent with desmoid fibromatosis ( I) SAN Q!EGO <Naval Medical Center> • llxtra-abdominal dcsmoid (fibromatosis) (9} YllNTIIBA • Fibromatosis (2) !.ONQ BEACH - fibromatosis (4) BAY AREA • Fibromatosis (3) MONTEREY <Community !iO!!nj!AI ofMontqey Penjnsu!a) • Fibroma (3); E:lastofibroma (I} BA!{ERSf!EID (San Joagujn Cornmyojtx HosPital! • Fibromatosis BAKllRSf!E!.O (Central Valley Study Group) • Myxoid leiomyoma HAYWARD CS~ RoseHospjta!l - FibromatOSis(4) NEV APA CRcnol - Extra-abdominal fibrom810Sis (desmoid tumor ) (2) IDAHO f!DX Pathologvl • Extra-albdornirull fibromatosis (dcsmoid tumor) CO!.ORAOO CNorth CoiO!Jld9 Medjrol CcnJcrl ·- Fibromarosis M!Ct!!QAN (foote Hospital! • Fibromntosis MICHIQAN 1St. Mary's Hospjl!l]l - 1•1bromatosis(desmoid)(4) NEBRASKA (Crs:jg!JtOnl - Benign fibromtliO!!is (extra-abdominal desmoid twnor) WISCONSIN IMcritcr Health Services! - Fibromatosis'''· myofibroma INDIANA (Goshen) - Benign fibrous tumor, fibromatosis I!.UNOIS - Leiomyoma NORDi CARQLINA IWNC Pt!thologyl - Extn-al>domina! desmoid tumOr (fibromatosis) (3) LOUISIANA (I grjsjana State Univmjw Medjcal Cent..-l • Dcsmoid (a~ve) fibromatosis D.OR!DA CTa!lahasseel - Extn-abdomina! desmoid tumor MABYLANDIWoodbinel- Leiornyomo(l); Fibrolipoma(l) MARYLAND fNationaJ Naval Mcdjcul Center\ • Fibromatosis (12} PENNSYI ,YANIA 0-ehigh Valley tlosniloll - Dcsmoid tumor (2): Fibromlltoois (desmoid rumor)(2); EJC!rn-abdominal fibromatosis

( I): Extra-abdominal desmoid (I) PJ\NNSY!.VANIAIConcmau•h Memorjal Hosoih~l - Fibromatosis PENNSYLVANIA !Lehigh Valley Ba.ni!ll!l • Dcsmoid tumor PENNSY!.VANIA IBB Group\ • Solitary fibrous tumor (fibromatosis) N£W YORK !Long .Island Jewish Medical Crotcrl · FiblOI!llliOli< NEW YORK !!mpathl - Low grodc fibromyxoid sarooma NEW YORK !Northponl - EJCtm.abdornina! fibromatOSis NEW JERSEY COm!"'* Hmnital) • FibromatOSis (3) MASSACI·lUSETfS lBctkshin; Modjeal Centql • Fibrom810Sis CONNF.Cf!C!IT <Unjv Conn !i!;l!hh Cguerl - J·ibromatosis JAPAN IKul'l!Shiki) - Oesmoid tumor (fibromatosis) (2) AUSTRA!.IA (Sydney} • Fibromatosis SAl/PI ARABIA (Kjns Khalid llnjvmjly Nospjta[l - Fibromato•is

DIAGNOSIS: FIBROMAT OSIS (EXTRA-ABDOMINAL DESMOID TUMOR), BACK

TYIIOO, M76100

R!lfERJlNCES: Allen PW. 1'he Fibromatoses. A Clinicopathologic Classification Based on 140 Cases. AmJ Surg Patho/1971; 1(3):255-270. Goellner JR tu1d Soule E!;l. Dcsmoid tumors, An Uhrnstructuml Study of Eight Cases. Hum f(Jiho/1980; II :43-SO. Ll M, Cordun-Cardo C, Gorald WC and Ro""i J. Desmoid Fibromatosis i• u Clonal Process. Hum Pat/w/1996; 27(9):939-943.

CASE NO. 2, ACCESSION NO. 27919

INLAND CRimi de/San !lqnardinol • Synovial sarcoma (2) ORANGE • Solitlll)' fibrou.~ tumor V AI..ENC!A · Hcmangioperic-ytomaiangiusarooma

APRIL 1999

SANTA ROSA • Soliwy fibrous rumor vs. hcmAAgiopericytoma ( l); Mesenchymal neoplasm consiStent with bemangiopcriC)1oma (l ): HemangioperiC)1oma (solitary fibrous tumor)(!)

SAN D!E('JO CNuynl Medical Center) • !lemangiop<.'l'iC)1omu (6); Solitlll)' tibrou.• tumor (3) VENTI!RA - Hemangiopcricytoma (2) LONG BEACH - Solitary fibrous tumor (4) BAY MEA - Angiofibroma (angiomyofibroblll!toma) (3) MONTEREY !Community t lospical ofMonta:ey Peninsula) - Neurofibroma (4) BAKERSFIElD (San looqujn Communjty Hospi!!!ll · Hemangiopericytoma BAKERSFIELD (Central Valley Studv Grounl • Leiomyosarcoma HAYWARD CSt. Rose Hoopit!!ll - Solitlll)' fibrous tumor (I); Vascularlumor (vs. sc:hwannoma) (I); Newofibroma(l); SclenJBin&

hemangioma (I) NEVADA {Reno) - Solitary fibrous rumor (2) IDNjO CIDX Pathologvl • Pelvic fibromatosis COI.()RADO (North Colorado Medical Ct;ntq) - Solillll)' fibrou.• tumor MICHIGAN CF001c Hospi!!!ll - Solitary fibrous rumor Ml CHIGAN fSt. Marv's HospjtaD • Solitary fibrous tumor (4) NEBBASKA (Creighton) • llemangioperiC)1oma WlSCQNSIN (Menter Health Sc'n'jeesl - Degenc111tcd peripheral nerve sheath tumor INDIANA fGoshcnl • Epithelial hemangioma ILLINOIS - Granular cell reaction NORTI! CAROLINA fWNC Pathology! - .Epithelioid hemangioendothelioma (3) LOUISIANA (f.oyjsiana Stg Univcrsjty Medical C!:nter) • Hemangiopcrkytoma n. neurofibroma Fl,()RIDA ITa!!!lhassce) - Myxoid neurofibroma MMYLANQ CW9odbjncl - Solitary fibrous tume< (2) MARYLAND CNaliO!U!I Nuy!l! Medical Center) - Solitary fibrous tumor (7); HemangiopcriC)1omu (5) PENNSYLVANIA<Lchii!h Valley Hoopjtall - Angiofibroma (l); Aggressive angiomyxoma (I); l' ibrous mesothelioma(!);

Hemangiopcric)1oma, benign ( 1): Favor solitary fibrous rumot (I); HemangiopcriC}1oma ( I) PENNSYt. VANIA fConemuugJ, Memorjul Hospjtall - Solicary fibrous tumor P£NNSYL VANIA flehji!h Valley Hoonjtall - Aw-cssive aoglomyxoma PENNSYLVANIA CBB GrouQl - Hcmaogjoperieytoma NEW YORK a.ong Is lund Jewish Mcdjgl Ccn!Ct) - Solitary fibrous tumor NEW YORK !lmJ!8thl - Aggrcosive angiomyxoma NEW YORK fNnr!hoort) • l!emangiopcriC)1omu ( I); Heman&ioendothelioma(l) N£W JERSf!Y «h'Sj!<lo!< ~l!!<l!itaJl - Hc'1llllllgjopcriC)10ma (2); fibrous rumor of tunica vugin!l!is ( I) MASSACHJ!SETIS Cllerbbire Mediwl Center> - ~lemangiopcriC)1omo (4) vs. ~lemangiocndothclioma (4) CONNECTICUT llJniv Conn Health Center) • Voscular tumor ofintermodiate mali &nancy, rio introm.,.culw hemangioma JAPAN fKurasbikil - Solitary fibrous tumor (2) AUSTRA!JA CSvdney) • Solitary fibrous tumour (6); Spindle ee!l 6pomo (2) SAUDI ARABIA eKing Khnlid Universi!y Hospi!!!ll • Pleomorphic hyulini1ing angicx."<:tatie tumor of soft purt

DIAGNOSIS: SOLITARY FIBROUS TUMOR, OBTURATOR REGION

TY2300, M9051 0

REfERENCES: Good lad JR und Fletcher CQ. Solitary Fibrous Tumor Arising at Unusual Sites. An!l!rsis of a Series. Histopolhology 1991:

19(6):515-522. Khalifa MA. ct a!. Solitary Fibrous Tumors. A Series of Lesions. Some in Unusual Sites. Soulh Med J 1997; 90(8):793-799. Va!lat·Dealuvelavc AU, Dry SM and Flclcher CD. Atypical and Malignant Solitary Fibrous Tumors in E><tratborncic Locations.

Evidence of their oom1)01abiliry to introthoraeic tumors. Am J Surg Patho/22(12):1501-1511, 1998.

CASE NO. 3, ACCESSION NO. 18414 APRIL 1999

!!!LAND IRiyqside!San Bqnardino) • Myxoid neurofibroma(!); Ncuro6broma wi1h myxoid chani\CS (1) ORANGE· MyxoidMHI VALENCIA - Myoma vs. myxoid lipa5aroomu SANTA ROSA • Myxoid Mf'H (3) SAN DIEGO !Naval Mec!lcal Canerl • l.ow grade myxofibromsarooma ( II) VENTURA • Low g11lde myxoid liposarcoma (2) LONG BEACH • Atypical myxoma (4) BAY AREA • Low grade Upc5arcoma (3) MONTERllY (Community llospilal ofM!MJterev l'enirn~u!al - Myxold liposaroomo (4) BAKERSFIElD (San J000ujn Gnmmunjty Ho:milal\ • Myxoid liposarcoma BtiKER,Sf!ELD CCcnua! Valley Srudy Gtpup) • Myxoma UAYWARQ CS1 Rn<c Hospjta!l • Myxoma (4) NEVADA (Renol • Myxoid lipc5arcomo (2) IDAHO IIOX Pathology\ • Myxoid liposarcoma COLORADO Worth CoiO!J!do Mediad Center> - Myxoma (large rumor, rule out malignant E. C. myxoid Mfll) MICHIGAN (fOOl!; Hosphall - Myxoftbn>san:oma MICWGAN CSt, MillY's tlospilall - Benisn myxoid neural neoplasm (4) NEBRASKA CCreighlon\ • Myxoid mnlitp1nnt fibrous hl,.iocytoma, low grnde WiSCONSIN CMeriter Heal1h Sgyjces) • Spindle oelllipoma \\ith prominent myxoid IC:alwes vs. myxoma INDIANA !Goshen) - Nodular filsciilis ILLINOIS • Ner;e .sbc8lh myxoma NORJJj CAROLINA IWNC Palhologyl • Benign myxoid prolifenuion, myxoid neurofibroma vs. nodular 18scii1is ( I); Nodular

fnsciitis (2) LOUISIANA floujsjana State University Mediad Cen!ql • M}?(oid lipc5an::oma, low grnde vs. myxorna FLORIDA !Tallahassee\ • Low grade myxoid nlalignant fibrous histiocytoma MARYLANQCWOO<lbinel- Angiomyxoma(I);Myxosarooma(l) MARYLAND !National Npyal Mediaol Cen1erl ·. Spindle eel.l lipoma (8); Myxoid nerve sheath 1arnor- (3); Myxold Mfll (I) PENNSYLY6NIA!l.ehjoh valley Hospil!!ll • Myxoma (4); Myxoid malignant fibrous histiocytoma (I): Juxra-anicular myxomo

( I) PENNSYL'fAt!IA (Cooqnaveb Memorja! Hosoitall • Intramuscular myxoma PENNSYLVANIA l!..chigh Valley Ho:!Djto!l - Myxoma PENNSY!.YAN!A fBB Grouol • Intramuscular myxoma NEW YORK !Long I< land Jcwis!J Medical Ccnler) • Juxuwortiad myxoma; Well-diftcrcntilllcd myxofibrosaroomu NEW YORK C!mooth) • Myxofibrosarooma. low grade NEW YORK 1Nor11lponl • Low grade myxofibrosarcoma (myxoid MFH) ( I); Low grnde fibromyxosorcoma (I) NEW JERS!)Y (Overlook HosniU!ll - Myxoma (3) MASSAOIUSE'ITS fficr!sshirc Medico! Center) • Juxlwticular myxomu (8): vs. netarothclioma CONNECTICUT {{Jniv Conn Health Cgll•t> • Low grade myxoid nlalignan1 fibrous histiocytoma JAPAN (KU!P.'fhikD • Myxoid sarooma. suggestive of MH I (2) AUSTRALIA (Sydney) • Myxoid malignant fibrous histiocytoma (7): Low grade myxoid fibrosarooma (I) SAUDI ARABIA !King Kholid Univqsilv llospilal) • Low grade fibromyxoid saroomo

DIAGNOSl S: LOW GRADE MYXOFIBROSARCOMA, SHOULDER

TYI220. M88403

CONSli!:CAIION: Madeline S. Milcbcll, M.D., Anmed Forces lnSlitUlcofPalhology. "Low grade myxofibrosarcoma."

REFERENCES: Men12el T. Edwnrdo C. Waddc:n C. Carnp!ejohn RS, e1 Ill. Myxofibrosarcoma Oinicopalhologic Analysis of75 Cases with

limphyxis on the Low·Gradc Varian~ Am J Surg l'ollro/1996; 20(4):391-405, E1•1U1S Iii .. Low Grnde l'ibromyxoid Sarcoma. A n:port of 12 cases. Am J Surg l'orhol 1993; 17:595·600. Kindblom LQ, ct al. lbe Ultrastructure: of Myxofibrosarcoma. A study of II """""- Virclrow Arch A PolhQI Anat Hislo 1997;

381:121-39

C ASE NO. 4, ACCESSION NO. 28141 APRlL1999

IM.ANP CRj..,ajdc;ISan !lqnaa!jno) • Dmnatofi~a prolubcnws (I); Malignant fibroul histiOC)1cma(l) OKJ\NGE - Leiomyosarcoma VAL6NCIA - Flbrosaroomn vs. myolibrosarcoma SANTA ROSA - Leiomyosarcoma (I); MCS01chymal {tumor of skin. nialigrnsnt, oon.•istcnt with leiomyosarooma rio AFX (I);

G.I.S.T. ofunlcnO\m malignant pot..,tial (I) SAN DIEGO fNoval Medical Center) - Leiomyuoarcoma ( 10); At)llical fibroxanthoma(l) VENTURA - At)llical fibroxunlhoma (2) LONG BEACH - Leiomyosarcoma (3); DFSP (I) BAY AREA - Leiomyosar<m>a (I); Spindle cell sarcoma. NOS (I); Spindle cell rflabdom)oosarooma (I) MONTEREY (Conunynitv ~losoi!al ofMontqey Peninsula) - DFSP (4) BAKERSFIELD !San Joaayjn Commynjrv t lospitall - Ckrmatoflbrosarcoma protuberans with nn:as r/o mruignant change BAKERSFIElD CCen(ral Valley Study Group) - Metustillic renal cell c:arcinoma HAYWARD <St. Ros Hospila!) - Atypical fibro.'Wilhoma (2); Leiomyosarcoma (2) NEVADA CRenol - Leiomyosarcoma (2) IDAUQ IIDX PqUJO!ogy) - l,.eiomyosareomu of vascular erigin COLORA!)() !North ColO!l\do Medical Centql - Leiomyosarcoma MICHIGAN IFogsc Hospital> - Al}pical fibroxanlhoma MICHIGAN lSI. Marv's Hmpjtal\ - Lciomyosaroomo (4) NfiORASKA CCrejghton) - Lciomyooaroomu WJSCONS!N {Mcritcr Health Seryice~~\ - Epithelioid lciomyooaroomu of skln INDIANA lGosbsp) • Sar<m>a J!.!.INQJS - Malignant. fibrous histiocytoma NORT!i CAROLINA fWNC Pathology\ - At)llical fibroxrutthoma (2); Spindle cell ma)jgorutcy? Fibrosarcoma/ al)llica!

fi broxanthoma ( 1) LOUISIANA (l.oujsjana State \lniversity Medjcal Centq) - Dennmofibrosar<loma protubctans FLORIDA !TaiiOOa«sccl - Epithelioid leiomyosara>ma MAim AND (Wqodbinel - Leiomyooarccma (2) MARYLAND INnlional Naval Medical Center) - Leiomyosarcoma (12) PENNSYLVANIA !Lehigh Ya!lcv HO$njtall - Fibrosarooma (fibroblastic) (I); Ocar cell sarcoma (I); Koposi's sarcoma (I);

Leiomyosarcoma (3) P!iMNSYL VANIA !Conemaugh Memorial Hospital) - Atypical fibroxanthoma PENNSYLVANIA Cl..ehjgh Volley Hosnimll - Oear cell sarcoma PENNSYLVANIMBB Group) - CtJtaneoos leiomyosarcoma NEW YORK O,gng Islan<l Jewj$11 Medica] Ccn!erl - Lciomyosan:omu NI\W YORK Clmoath) - Fibrooarcoma NHW YORK CNorthoon\ - LciomyosiU'COrna NEW JERSEY (Overlook HospjtaJI - Cutaneous leiomyosarcoma (3) MASSACHUS!ITJ'S !1lc:!k:!IJire Medical O:nterl - AFX (8) CQNNECTIC\l[ Q[niv Conn Health CcniL1) - At)llical fibroxanthoma, rio spindle cell squamous cell COJ'<inoma or melanoma It\!' AN CKurashjk.j) - Myofibrobla.~ticsorooma.low·&rndc ( I); Fibrosan:oma (I) AUSTRALIA £Sydnevl - Leiomyosarcoma SAUDI ARABIA !King Kbaljd Univqsi!V Hoopital) - Sorooma

DIAGNOSIS: CUT A!VEOUS LEIOMYOSARCOMA, CHEEK

TY0300, M88903

REfliRENCI'.S: Dahl I and Angervoll L. Cutaneous rutd Subcutaneous Leiomyosarcoma. A Clinicopathologic Study of 47 PatientS- Pntlrol 6ut

1974; 9(4):307-315 . •• Sustcr S. Epithelioid Leiomyosorcoma of the Skin and Subcutaneous lisstJe. Oinieopathologlc. Immunohistochemical. and

Uhra'<trucmrnl Study of Five Cases. 11111 J Surg l'atho/ 1994; 18(3):232-240. Fields JP and Helwig L>6. Lciomyosaroomu of the Skin and SubcutMcous lissuc. Cancer 1981; 47( 1):156-169.

CASE NO. 5, ACCESSION NO. 28297 APRIL 1999

INLAND (Riverside/San Oemardinol • Gastrointestinal stromal tumor of uncertain malignant potential (2) QRANGE • Leiomyosarooma · VALENCIA • GJ.S.T., lci<>myosarooma SANTA ROSA · G.l.S.T. ofund"!ennined malignant potential (3) SAN DIE\J{) <Na>'Bl Medical Center) ·Malignant G.LS.T. (11) VENTIJRA • Smooth muscle tumor of uncertain biologic potential (2) LONG BEACH - Smooth muscle tumoroflow malignant potential (4) BAY AREA • Low gradclciomyosarooma (2); Leiomyoma (epithelioid type 'I) (1) MONTEREY (Community HospjcaJ of Monterey Peninsula) • G.LS.T., malignant (4) BAKERSFIELD <Sao Joaquin Communitv Hospital) - Stromal tumor ofGJ tra.:t, malignant (leiomyosarcoma) BAKERSFIELD (Central Valley Study Groug) - Fibrosarooma HAYWARD (St RO!i!l Hospital) • GJ stromal tumor, G.I.S.T. leiomyosarooma type (4) NEV ARA (Reno) - Leiomyosarcoma (2) IDAHO CIDX Pathology) • Angioleiomyoma COLORADO (North Colorado Medical Center) : G.l.S.T .. smooth muscle <ype, borderline malignam MICHIGAN {Foote Hospital) • Malignant g~~Stroimestinal strOmal rumor MICHiGAN CSt Mary's Hosoi!!l)l - Epithelioid leiomyosarcoma (4) NEBRASKA (Creighton) • Malignant G. I. stromal twnor WISCONSIN (Meriter Health ScTVicesl - G.l.S.T .. smooth muscle type,,malignam:(l2 em}, (L"'S) INDIANA (Goshen) • Epithelioid leiomyosarcoma (stromal tumor) JLLJNOIS - Small intestinal stromal tumor, low grade malignancy NORTH CAROLINA fWNC Pathology) - Gastrointestinal stromal tumor (2); Smooth muscle tumor of uncertain malignant

potential (I} . LOUISIANA <Louisiana State Univcrsjty Medical Center) • G.J.S.T, (g~~Strointcstinal stromal tumor), malignant FLORIDA <Tallahassee) • Leiomyosarcoma MARYLAND (Woodbine} • G.I.S.T. (I); Epithelioid leiomyoma ( 1) MARYLAND <National Naval MedjcaJ C,enterl • Gastrointestinal siromal tumor with smooth muscle differentiation of uncertain

malignant potential PENNSYLVANIA 0 &hiah Valley Hoopitall - Leiomyosarcoma (I); Gaslrointesiioal strOmal tumor of uncertain malignant potential

with features favoring malignancy.(3); Malignant stromal tumor at small bowel (I); GastrOintestinal stromal tumor (I) PllNNSYI.YANIA (Conemaugh Memorial Hosoitall - Leiomyosarooma PENNSYLVANIA (J.ehigh Yallcv Hospital) - Gastrointestinal stromal rumor of uncertain malignant potential with features

favoring malignancy PENNSYL V ANlA CBB Group) · LeiOmyosareomll. low grade NEW YORi< (J.ong Island Jewish Medjcal Center) - Leiomyooarwma NEW YORK Omoothl • G.J.S.T., likely malignan~ with myxoid differentiation NEW YORK <Northoort) • G.I.S.T .. smooth muscle type, borderline t.'"EW .JERSEY (Overlook Hospital) - G.i.S.T. (probably Jeiomyosarooma, low grade) (3) MASSACHUSETTS <Berkshire Medical Center) - G.J.S.T.;typc I, bordcrline(3); Malignant (4} CONNE¢TJC\IT (!Jniv Conn Health Center) • Malignant gastrointestinal~tromal tumor with smooth muscle dill'erentiation JAPAN (Kurashiki) • G.J.S.T., with smooth muscle djfferentialion, borderline (2) AUSTRALIA (Sydney) •· Ga•trointestiilal stromal tumour with smooth muscle diffi,..iOtiation SA[JPI A~ABIA /Kino Khalid Univcrsitv Hoopitall - Malignant gastrointestinal stromal tumor of smooth muscle origin

DIAGNOSIS: LOW GRADE LEIOMYOSARCOMA, SMALL BOWEL (Director's Note: See I~ reference below)

T64000, M88903

REFERENCES: Miettinen M, Virolainen Mt and Sarlomo-Rikala M. G11SlrOinte..OnaJ Stromal Tumors. Value ofCD34 Antigen in Their

Identification and Separation From True Leiomyomos and Schwannomas. dm .I Surg Patho/1995; 19(2):207-216. Min KW. Small Intestinal Stromal Tuniors with Skeinoid Fibers. Clinioopatho!ogical,lmmU(IOhistochemical and )Jitrastrucwrdl

Jnvestigntions. Am .I Surg Patho/1992; 16(2}:145-155. Ng EH, Pollock RE, Munsell MF. et al. Prognostic· factors Influencing Survival in Gastrointestinal Lelomyosaroomas. Implications

foe Surgical Management and Staging, Ann Surg 1992; 215(1):68-77. Ricci A, Jr., Cicearclli 0 . Canun RW and Newcomb P. A CJinioopathologic and Immunohistochemical Study of 16 PatiollS with

Sm•lllntestinal Leiomyosarooma. Limited Utility ofln1munophcnoLyping. Ccmcer 1987; 60(8):1790·1799.

CASE NO. 6, ACCESSION NO. 27886 APRIL 1999

INLAND (Rh·ersidc/San Bantvdinol - Well-difl'erentiatcd liposiU1XJma (atypical lipoma) (1 ); Atypical lipoma ( 1)

OBANGE - Wcll-difl'cn:ntia!cd liposarcoma VALENCIA • Liposarcoma SANTA ROSA - Wcll-differcntiDicd liposarooma (3) S4N DIEGO <Naval Medical Center) - Well-differcntialed lipo$11l1)0ma. S<lcrosing subt)pe ( 11)

VllNTIJRA - Low srndc liposlli'OOma (2) LONG BEACH - Sclerosing liposarcoma (4) BAY AREA - Wcll-di ffete01i81ed sclerosing lipo$11l1)0m8 (3) MONJEREY <Community HO<njiAJ of Monterey Psnin<ulal • Welkliffcmuiased liposarooma (4) OA!(ERSf!Bl .D /San Jouquin Communiw llo.<nitall • Liposarcoma, well-differentiated 8AK£RSFIEW <Cen11111 Valley Study Grpuol • UpoSl111)()fl1a HA YWARJ) 1St RO<e tlowil!lll - Acypicallipoma (AKA), low grade liposan::orna (4) N!lV ADA CRcnol - Well-differentiated liposOli'OOmll, sclerosing type (2) IDAHO C!DX Patho!O!I\') • Well-differentilllcd liposarcoma COWRADO !North Col!lfl!do Medjca! Ccrcql - Wc!l-diffcreotiated liJIOS3COOOID (81ypical lipoma) MICHIGAN (foote Hospital\ • Well-differentiated liposltfOOma MICHIGAN (St MillY'• Hosnj!OJ\ - Well-dilferentiatcd l.iposarcoma (3); Pleomorphic liposlli'OOma (I) NEBRASKA 1Crei¢uoo\ - Uposarooma. wcll-diffcreoti81ed WISCONSIN !Mcriter Health Sqvioesl - Well-diffacntiatcd liposaroorna vs. atypical lipoma INPIANA <Goshen\ • WcU-diffi:rcnliatcd sclerosing liposW'OOma ILLINOIS • Well-dilfcrentillled liposarcoma NQRTH GAR OLIN A 1\VNC Plllhologvl - WeU-differmtiated S<:lerosing liposarcoma (3) LOUISIANA <kouisiana State Upjversity Medical Center} • Low grade liposarcoma vs. Dlypical lipoma fLORIDA CTa!lahasseel - Lipo$011'00ma MARYLAND !Wooclbjncl - Well-<liffet~."Ptialed S<lcmling lipo$0li'C0"'8 (2) MARYLAND (Nationol Naval Medical Center\ ·.Sclerosing liposarcoma ( 12) PENNSYLVANIA ([&high Valley Hospjtnll - Fibrolipoma vs. wc!l-<liffcr<:ntiated lipoma ( I); WeU-<Iilli:n:ntiatod llposarcom~ (3);

Myxoid liposarooma ( I); Uposarooma (I) Pf.NNSYLVANIA <Concrnauab Memorial Hospitn!) - Ple<lmOrphic liposarcoma l'!lNNSY!.\fANIA IU:hjsh Valley Hospital) • Wcll-differcntintod liposorcoma PENNSYLVANIA CBB Grouol • Wcll-diffen:ntiDied Klausing lipos.arcorna NEW YORJ{ {Long Island Jewjsh Medical Cmterl • WeU-diffcrmtiated liposarcoma NEW YORK flmnath\ - Sclerosing liposnrcoma, low grade NEW YORK <Nonhoort} • Pleomorphlc lipoma(!); Low grade lip0S111'Cl011la (1) NEW JERSEY {()yttlook HO!!pjti!ll - Lipo$11l1)0ma, wcll-diffCTCPtiated (3) MASSACHUSETTS lllerlcihin; Medical Center) • Atypical lipoma (3); w.c!l-<liOcrcntiatcd lipo$11l1)0ma (5) CONNECriCUT fUojy Conn Health \.entcrl - Wcll-diffen:ntiatcd lipo•llrooma JAPAN <KUG\hikil - Well-differentiated liposarooma (2) AUSTRALIA <Svdncyl • Well-differentiated sclcro•ing lipo$011'00ma SAUDI ARABIA <King Khalid Univer<ily Hospital\ • Sclel'O!Iing (well-difl'etentiated) Jipoourooma

DIAGNOSIS: WELL-DIFFERENTIATED SCLEROSING LlPOSA,RCOMA, INGUINAL REGION

TY7000, M88503

REEERENq;s: LUCII$ DR, Nascimento AG, SW.joy BK and Rock MG. Weii-Oiffcrcn!iau:d Uposorooma. The Mayo Clinic Experirooe with 58

Cases. Am J Clin Pot/ro/1994; I 02(5):6n-683. Dalla Palma P and Bnrbal:ia'R. Well-Differentiated Liposarcoma of the P:u-atesticulur an:o. Rcpon of a Cuse with Fine-Needle

Aspil'lllion Preopcntive DillgllaSis and Review c>fthe Literature. Dlag C,topatho/1990; 6(6):421-426. Farshid 0 and Weiss SW. Massive Localized Lymphedema in Morl>idly Obese. A Histologically Distinct Reaaivc J.,esion

SimulatingLip<>Sarcoma. 1/m J Surg Potho/1998; 22(10):12n-1283.

CASE NO. 7, ACCESSION NO. 27968 APRJL 1999

INLAND CRjvmide/San Bernardino> • Malignant peripheral ne.ve sheath twnor (I); NeurofibrosataJcrul ( I) ORANG£ - MPNST, low grade V AlllNC!A - Now-ofibrooarooma with OSSIOOU$ metaplasia SANTA ROSA • Malignant peripherol n1.-rvc sho:uth tumor(!); Malignant mcse:nchymal neoplasm consiste-nt with neural origins,

reoommcnd II'OX (I); Cellular neurofibnlmo, rio peripheral nerve sheoth snroomn (I) SAN P IE(){) CNaval Medical Center\ • Malignant peripherol nerve sheath tumor (II) VENTURA - Neurofib<osar<:omn (2) LONG BEACH • Neurosarooma (4) BAY AREA • Neurofito'osarooma (3) MQ!flEREY ICommunirv HosPital ofMQ!l!Cm Peninsula) - Neurofibrosarooma (4) BAKESSEIELO <San Joaqujn Community HQ!Ipitall - Neurofibroma BAKERSFIELD <Cenva! Valley Stycly Gmupl • Schwanooma HAYWARD ISL Rose Hospital\ · Neurofibrosarooma (4) NEVADA CRenol • Malignant peripheral nerve sheath tumor with osseous mcu.tplnsia (2) !J)AHO CIDX Po!holocyl • Mcta.5tatic malignant neurofibroma (with areas of post-rodiadoo ostl!()$8rCOma) COLORA!)O CNonh C01ora4o Medjg!l Cente-r\ - MPNST with heterologous clements MIC!ii{)AN (Foote Hospital> - Mnlignunt pcriphcrnl nerve she01h rumor MICHIGAN ISL Mll!'y's Hospital\ • MllllgnMt peripheral nerve shelllh turnor (4) NERRASKACCrciehton) - Neurofib<osarooma WISCONSIN CMeriter Hcallh Saviocsl • Neuro6brosarooma and ostrosaroom.a (mali87'ant mesenchymoma) !NQlANA <Goshen) - Malignant schwannoma !LLfNOIS • Ncuro6bromalosis with molignant1l1111Sfonnadon (MPN$1) NORTII CAROLINA IWNC Pa!hologyl • Malisnant peripheral ncrvt: sheath twnor (2) LOUISIANA C!.ouisiana ~ate Uniymitv Medical Centerl - Malignant nerve sheath tumor, mali87'nnt fLORIDA ITp!lnhassccl • Malignant peripheral nerve sheOih tumor MARYLAND !Woodbine) • Ncurolibrosarooma (2) MARX!.AN!) !NutiQ!la! Naval MediCA! Cen!ql - Ma!\gnant peripheral ncrvo sheath tumor (I 2) PllNHSY!.\'ANIA l!..ehich Valley Hosojud\ - Mali81'3nt collag<:nous schwsnnoma (or neuro6bro$arcoma or oollugenous

neurogenic 7) (I); Malignant peripht:nd nerve sheath tumor with focal ost""""'"""'a (I); Malignant schwannoma with heterologous clcrncn!S (!);Peripheral nerve sheath tumor (I); Malignant periphcrnl nerve sheath rumor(!); Neurogenic sarcoma (ncurofibrosarooma)

PENNSYLVANIA !Thnemal!!!h Memorjll Hospital! · Neurofibroma with oss-.s IDCI:lplasia PBNNSYl.VAN!A Q.ebigl! Valley HosojJaJ) - Malignant peripheral nerve sheath tumor with focal mteosarcoma PENNSYLVANIA fBB Gmuol • Neurofibrosornoma NEW YORK <Lon• Island Jewjsh Medjgl Center) - Malignant mesenchymoma of nerve sheath with he!croloaous clements NEW YORK l!moath\ - Malignant pcriphcrul nerve shelllh tumor N!\W YORK (Nonhport\ · Malignant pcrlphcml nerve sheath tumor NEW JERSEY (Overlook lfosoital) • Ncuroflbrosarooma (3) MASSACI tl!SBTTS !BerJsshire Medical COmer) - Malignant peripheruJ nerve sheath tumor CONHECIICUI fUniv Coon Health Center) - Metastatic osteOgenic •arcomu IAPt\N CKurashikil • Malignant peripheral nerve sheath lumor (2) AUSTRALIA (Sydney) - Molignant peripheral nc:r.-e shea1h rumour (neurofibTOSara>ma) SAUQI AMBIA (Kjng Kltalid Uniymily HosPital\ - Malignant peripheral ncm: sheath tumor

DIAGNOS IS: MALIG NANT PERIPHERAL NER VE SHEATH TUMOR, MEDIASTINUM

TY2300. M80001

CONS\JJ; I'ATION: Sharon Weis.•, M.D., Emory University. "Malignant •chwannoma." She felt th31 the material Wtl$ metastatic rather than rcl!e"1ing a seOOild primwy and also that the tumor wa.• high pde.

Rlifl'lR!lNCES: Moon WK. lm JG, Ham MC. Mali87'ant Schwonnoma of the: Thorax. Cr Findin31- J Comput Assist Tomogr 1993: 17(2):274-276. Pelton J, J!ldckola S. Lebwohl M, Renva!l S, d al. Cellular Differentiation and Expression ofMOirix Genes in Type I

Neurofibromatosis. lAb lnvnt 1988; S9(6):760-nl. Un BT. Weiss !.M, Medeiros U. Neurofibroma and cellular neurofibroma with atypia: A n::pon of 14 tumo<s. Am J Surg Pothol

21(!2):144J-1449, 1997.

CASE NO. 8, ACCESSION NO. 27888

INLAND (Rjvcrnidt/San Bem!!!dinol - Anaiooarooma (2) ORANGE - Anglosnrcoma V ALEJ:IC!A - Anaiosarooma r/o mdaStaSis from papilhuy carcinoma of th)Toid SANTA ROSA - High grade angiosan:oma ( I); Angio<an:oma (2)

APRIL 1999

SAN QIEGO CNaynl Medical CC!Jtc<l • Culaneous Mgiosnrcoma (I); Epithelioid nngiooaroomn (10) VENTURA • An&iosnrcoma (2) LONG BEACH - Angiosarcoma (4) BAY AREA - Angiosatcoma (2); Papillary can:inoma, NOS (I ) MONTEREY !Communi!>' Hosoilal ofMonu:n;y Peninsu!!!) - Angiosorcoma (4) BAKERSPIEW C$110 Joauujn Cgmmunjty Hosoltall • Angiosarcomu BAKERSFIELD CCcntral Valley Studv Group) • Angiosatcoma HAYWARD (SL Rose HOS!lita!l • Angiosarcoma (4) NEVADACRcno) • Angiosarcoma (2) !DAJo!O CJDX Pa!lJologyl • An&losatC<Jma COLORADO CNonb Colornd<> Medical CeDJql - Angiostlrcoma MICHIGAN CF001c tfomitall • Aogiosan:oma MICHIGAN CSl Mwv's HMpita!l • AnglosarC()ma (4) NEBRASKA (Crelsbtonl - Angiosarcoma WISCONSIN CMC!jtcr Health Sqyjoesl • Angiosarooma IN[)IANA ([..,.hen) • Ah-oolar rbabdom)'OSllt'OOIDll ILLINOIS • Angiosarooma (3) NORTH CAROLINA CWNC Pl!thologyl • Angiosarcoma (3) L..OU!SlANA<Loujsjana State \Jnivcrsjty Medical Cemer) - Alveolnr rhabdomyosarcoma vs. acinic cell carcinoma F!.ORIDA ITa)!!!hnsw) - AngiC>Sal'(()ma MARYl.AND !Woo4binel • Angiosarcoma (2) MARYI..AND (National Naval Medical \-"fltcr) -. Angiosarcoma ( 12) PENNSY!.VANJA (!.shish Valley Hospital) • Nasopalatine cyst ( I); AngiO<>arcon1a (5) PENNSYLVANIA IConcmauf!b Memorial Hospital) • Angi051U'001T1& PENNSYL VAN) A C!.cbjo!J Valley Hospiti!l) - Angio.<an:oma PENNSYLVANIA UlB Group) • l'apillary angiooarcomn NEW yORK O..Ona Island lewisb Medical CC!ltcr) • Angiosarcoma NEW YORK Om!!Bih} - Angi~a NEW YORK £Northport) • Ansiosaroomu NEW JliRSEY IOyerlook Hoollill!!} • Angiosarcoma, Jll'Ude 2 (3) MASSACHUSETTS IBerkshin; Medical CC!Jierl • An&iosnrooma CONN!;cT!aiT Q)niv Conn !1ca!th Centql - Angiosanxma JAPAN CKvra•hi!cjl • AngiOBarooma y,ith rhabdoid fcat...:s (2) AIJSTI~ALIA CSydnevl • Angiosurooma SAUl)[ ARABIA IKing Khul jd \/nlvqsjry ll<"pitall • AngiosarC<Jma

DIAGNOSIS: ANGTOSA.RCOMA (Grade II of ill), MAXILLA AND HARD PALATE

'1'10170. M91203

RF.fERENCE.<;: Fu Y -S ond Perlin KH. Nonepithelial Tumo~ of the Nasal Cavity, Pttnll1aSa! Sinu.'!CS and N~m. A Clini""fl'llhologjc Study

XI. General l'eatures and va.,cular Tumors. Cancer 1974; 33(5): 1275-1288. Heffner OK. Problems in Pediatric Parbolol:Y II. Vaswlnr Tumors and Lesions of the Sinona.al Tract and Nusophllr)11X. lnt J

Pt diotr Otorhlnoloryngic 1983:5(2) 12S· I38. Kimura Y, T3!1!lkA S andl'brulcawa M Angiosarcoma of the; N:lsal Cavity. J Loryngol Oto/1992; 106(4):368-369. Kuricn M, Nair Sand ThomiL• S. Angiowooma of the Nll:lal Cavity ond Ma:dllary Antrum. J l.oryngo/oto/1998; 103(9):874-876.

'

CAS£ NO. 9, ACCESSION NO. 28338 APRIL 1999

11\'LANP (Rive!J!jdc/San Bsroardinol • Ewins's sarroma (I); Primitive neuroendocrine tumor (Ewing's) ORANQE • PNET VAL!lNCIA · !!wing's sarcoma vs. PNET tumor SANTA ROSA • PNET (2); Small round-Cell neoplasm consistent wilh periphoral neurooetodermul tumor (I) SAN DIEGO {Naya! Medjc:aJ Ccn!et) • PNET (10); Extraskdctal Ewing' s/PNET(I) \'EN1URA · Ewing's SotWma (2) WNG BF..ACii • Ewing's(4) BAY AREA • Ewing!PNET sarcoma (3) MONTEREY CCommWlity Hospj!al of Monterey Peuinsulal - PNET (4} BAKijRSFIELD CSan Jo!l!!ujn Community Hoopjtall • Extra.•kelctal £wing'• sorooma BAKERSFIELD (Central Valley Study Grounl - Ewing's sarcoma HAYWARD CSL Rose H00pitall • Ewing's t~ (also c:aJied malianant small cell tumor of the thoracopulm<lOOI}' ~on) (4} NEVA!) A CRcnol • Primitive ncuroe<~odcrm"l tumor/E><tro·skelctoJ Ewing's sarooma IDAHO CIDX Palhologvl • Small round cell saroomac/w Ewing's COI.QRADQ CNO!th Co!O!pdo Mcdjcpl Ccn!ql • PNET (Askin Twnor·likt p-esentation) MICHIGAN Cfomc HQSpjtall • Desmoplastic small round cciiiWl\Or MICHIGAN CS!. Mncy's Hospital) • Askin tumor (PNE'I) {4}

NEBRASKA CCrsi!!hl!lnl - 6xlraskdctal£wins'• sarcoma WISCQNS!N (Mcriter Health Services) - Ewing's sarcoma INDIANA (Goshen) • ExtrUskeletal llwing's sarcoma l!d.INO!S - Extraskeletal Ewing's sarcoma NORD! CAROLINA CWNC PatlloJogyl - Extra<kelctal Ewing's/PNET(l} LOUISIANA (Loyjsiana Stnte Univmjty Mcdjcnl Center) • PNET (Askin tumor) FLORlDA ITall!lbasseel • E"ing's sarcoma; I'NET MARYLAND (Woodbine) • PNET (I}; Ni:uroendocrine .,.,.a noma (!} MARYLAND <National Nayal Medjeal Ccnler) •• PNET ( 12} PENNSYLVANIA CJ.ehigb Valley l·loopjtaD • Ewing's sarooma (3); Extraskelctal Ewing's sarcoma (2); Smllll round blue cell

mali~x1r8Skeletal Ewing's sarooma ( I} PENNSyLVANIA CConcmnush Mcrnorial H01pj!Jl!l - Extraskeletal E"ing's sarcomo!PNET PENNSYLVANIA O..Chjgb Valley lio~pitall • Extraskclctal Ewin&'s sarcoma PENNSYLVANIA fBB Grouol - Extmsk:eletal Ewing's NEW yORK llono Island Jewish Msdjca! Center) - Ewing's sarnoma (diffe>1:ntial diagnosis includes small c:ell95teOSOrWma} 1\'EW YORK llmnathl - Ewing's sarcomo!PNI!T NEW YORK Q!or1l!por!) • Exuaskclctal Ewing's sarcoma/PNET NEW JERSEY COver!ook Hwpitall • Ewing's sarooma (3) MASSACHUSETTS fBer!Qhire Medlcul Ce!>tcrl - Merkel ceO carcinoma (2) vs. Ewing's sarcoma (4); vs. PNI:."T ( I); vs.

Desmoplastic round cell tumor ( I) CQNNECTJCI!f 0 !njv Coon Hc:aJ1h Center) - E"ing's sarooma JAPAN Ct<urashildl • Desmoplastic small round cell tumor (2} AUSTRALIA CSvdney) • Primitive ncuroectodcnnBitumour ofthoruoopulmonru-y origin (Askin's tumour) (6), Ewing's sarcoma (2) SAUDI ARABIA !Kino Kba!id Univqsity Hospjta!l - Peripheral neiU'OC®denna! tumor (PNET

DIAGNOSIS: PRIMITIVE NEUROENDOCRINE TUMOR (PNET/EWING'SIASKIN), CHEST WALL

TY2100, M.92603

REFERENCES: Zucman I, Melot T, Dema7.C C. e1 al. Combinational Gencrution of Variable Fus ion Proteins in the Ewing Family of Tumors.

EMBOJ 1993; 12(12):4481-4487. Ladanyi M. The Em<Tgin& Molecular Genetics of Sarcoma TransiOCIIIions. Dfag Molteular Patho/1995; 4(3):162-173. Schmidt D, Herrman C. Juracns Hand Harms D. Malignant Peripheral Ne-uroectodermal Tumor and its Neccs:wy DiSiinc:tion from

Ewing's Sanx>ma. A Rc-pnrt front the Kiel Pediatric Tumor Registry. Cancar 1991 ; 68(1 0):2251-2259.

CASE NO. 10, ACCESSION NO. 28367

INLAND IRivcaid!:/San Banardinol - Chondroma of soft potU (2) ORANGe - Myxoid lunl<lr. NOS. probably low grade VA!.IjNCIA - Extraskclctal chondrosarooma SANTA RQSA - Extraskclctal m)'Xochondroma {I); Chondroma of soft ti.ssoc (2) SAN QIF.C,Q IN3!j!l M!!dical Center> - Extrmkclctal myxoid ehondi'O!Ot<XlC1ta(ll) VllN'TIJRA - Lipoma with cartilaginous metaplasia (bcni&n mcscnchymoma) (2) LONG BEACH - M)'Xoid chondrosarcoma (4) BAY AREA - Cystic haroartoma with myxochondroid feature:! (3)

APRIL 1999

MONTEREY /Communi tv Hospital of Monterey Penjosylpl - Chondroawwma {3); Chondroid mc:taplosia ( I) BAKERSF!fiLQ /San Joaquin Community Hoonjlllll - M)'X<)id cbondrosnrcoma BAKERSJIIfl!..D /Central Valley Study \o<oup\ • Myxochondroma HA YW ARP ISL IInse Uospital\ - Extraskclctal cbondrosnroomo ( 4) NEVADA !Reno\ - Chondroid lipoma (2) IDAHO OpX l'a!hologyl • Chondroma-partially Dect<>tic COLORADO fNonh Colorado Medical Cattal • Exuaskelctal myxoid chondrosarcoma (chordoid sarcoma) MICHIGAN !foote Hospital\ - Chondroma of soft part

MICHIGAN 1St- MA!Y's Hospital) - Extraskeletal myxoid ci>ondrowooma (4) NEBRASKA (Crcjlll!ton\ - Extraskdctal myxoid c:bondrosarcoma WISCQNSIN CMcrjter Hca!tb Scryi(:CS) - E.xnaskclctal c:hondrosarooma INDIANA IC0hcol • Myxoid <booclrosarooma IlliNOIS • Cboodroid sarcoma NORTH CARO!.!NA IWNC Pathology) - Myxoid chondroaarooma (2); Extrmkcletal chondrosarcoma!Myxoid chondrosarcoma LOUISIANA !I .9!Ji!IMa State l!ojyernitv Medical Ccotq\ • Chondroaarcorna FLORJDA CTal!ahtWSeel - M)'Xoid chondroaarcoma MARYLAN)) IWoo<lbinc) • Chordoma (2) MARYLAND CNutjooal Navol Medical Ccntq\ ·- Extra.•kclctal chondrosaroeoma ( I I); Mixed rumor ( I) PENNSYLVANIA ll&high Valley Hospital) • Soli myxoid fibroma with areas of calcification and necrosis (I); O>ordomn ( I);

Myxoid chondrosarcoma (I); Myxoma (of nerve sheath?) ( I); Favor cxtraskeletol chondrosarcomn ( I); Myxomu ( I) PENNSYLVANIA (Conemaugh Memorial Hospjta!l - Wcl!-<liiTcrcntiatcd sol\ tissue myxoid chondrosarcoma PENNSYLVANIA (Lehigh Valley llospitall • Chordoma PENNSYLVANIA CB!l Groupl - Low grade chondrosarcoma NEIV yORK I! .!lOs !•land Jewish Medical Center) - Ext.raSkeletal myxoid chondrosarcoma NEIV YORK Omooth) - O>ondroma of soft parts,? unocrtain mali&nant potcntiol NEW YORK 1Nonhpo!1l • 6xttaskclctal chondtosarooma NEW J!lRSEY IOvcr!ook Hosoitall - Soft tissue c:hondrosarllom (low an>dc) (2); Myxoma (I) MJ\SSAQWSEJTS m..whire Medical Ccntcrl - Chcndroma (2); vs. Chclndrosan:oma (6) CONNECTICUT aJniy Conn Health Cent<l'l - Chondroid syrinpna (mixed tumorYchoodroma JAPAN (KI1!l!Sbj!cil • Myxoid choodrosan:oma (1): Extmkclcllll choodroma (I) AUSTR.ALIA !Sydney) • Extraskclctal m)'Xoid chondroowooma (7); Soft tissue chordoma ( I) SA LIP I AMBIA IKing Khalid Unjversity Hosnital\ - Myxoid chondrosarcoma

DIAGNOSIS: IJOW GRADE CARTILAGENOUS NEOPLASM, CONSISTENT WITH EXTRA·SKELET AL MYXOID CHONDROSARCOMA, RIGHT BUTTOCK

TY 1600, M92203

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Long-Tcnn Follow-tip. Cancer 1992; 70(12):2827-2830. Sci01 R. Dal Cin P. Fletcher C, et al: t(9:22Xq22-JI;q 11-12) is a Consista:>t Markc:rofExtrnSI::elctal M)'Xoid Chonclmson:oma.

Evaluation ofThnx Cases. Mod Pothol 1995; 8(7):765-768. Ahtamovicl LC. Stcina QC and Bonar F. M>"oid Chondroaorcoma of Soft 1issue and Done. A Retroopective Stody of I I Cases.

Hum Patho/1995; 26(1):1215-1220. Brody RJ, Ueda T. Jhanwar SC, Bridge JA, Henley Jll, t luvos AO, Gcruld WL and ladan)i M. Molc:<;ular Anal )'Sis of the Fusion of

EWS to'"' Orphan Nuclear Receptor Gene in Extroskeletol Myxoid Chondrosarcoma. Am J Patho/ 1997; 150(3):1049·1058.