CALIFORNIA TUMOR TISSUE REGISTRY

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

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

(909)" 824-4788 FAX: (909) 478-4188

1121

Target audience: Practicing pathologists and pathology residents.

Goal: To acquaint the participant with the histologic features of a variety of benign and malignant neoplasms and tumor-like cOnditions .

. Ob!ectlve:

The participant will be able to recognize morphologic features of a variety ofbenig!l and malignant neoplasms and tumor-like conditions and relate those processes to pertinent references in the medical literature.

Educational methods and media: . Review of representative 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

CMECred lt: The CTTR designates this activity for up to 2 hours of continuing medical education. Participants must return their diagnoses to the CTTR as documentation of participation in this activity.

Accreditation: The California Tumor Tissue Registry is accredited by the California Medical A~sociation as a provider of continuing medical education.

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

TISSUE FROM: Left neck

CLINICAL ABSTRACT:

'l .

CASE NO. 1 - MAY 1997

ACCESSION #28031

This 80-year-old female presented with a left-sided neck mass which did not appear to enlarge ·over the next six months. A left radical neck dissection was performed.

GROSS PATHOLOGY: The 240 gram, 12.0 x 12.0 x 5.0 em left neck contents specimen was largely replaced by tumor.

A 10.0 x 6.0 x 2.5 em portion of firm tan tumor extended from the midline into the lateral neck, apparently replacing the thyroid which was riot identifiable. A 4.0 x 4.0 x 3.0 em area of tumor was wrapped around the trachea and portions of the larynx.

CONTRIBUTOR: Robert E. Riechrnann, Jr., M.D. CASE NO. 2 - MAY 1997 Covina, CA

TISSUE FROM: Right mandible ACCESSION #28036

CLINJC.i\L ABSTRACT: This 29-year-old male presented with an expansile mass of the right mandible. Radiographic and

CAT scan examination revealed a 4.5 x 4.0 em mass involving the entire right ramus and partial body of the mandible.

GROSS PATHOLOGY: A 10 x 4 x 3 em curved, crescent-shaped piece·of hard white bone was expanded in the

·midportion by a 7 x 4 x 3 em mass composed of homogeneous dense white tissue within which there was an irregularly-shaped area of central cyst formation.

• CONTRIBUTOR: Chisa Aoyama, M.D. CASE NO. 3 - MAY 1997

Sylmar, CA

TISSUE FROM: Pelvic mass ACCESSION #28210

CLTNICAL ABSTRACT: This 21-year"'Old female presented with a 4 month history of intermittent episodes of

· diaphoresis, headaches, blurred vision, shortness of breath, palpitations and fatigue. Her blood.pressure during physical examination was elevated (as high as 203/116) and labile. Twenty-four hour urine catacholamines showed normal levels of epinephrine with markedly elevated levels of norepinephrine, VMA and dopamine. cr scan of the abdomen and pelvis showed a large mass in the pelvis behind the uterus. The mass was resected along with a portion of adherent sigmoid colon. There were multiple nodules in the omentum and mesentery.

GROSS PATHOLOGY: A 11.5 x 8.5 x 7.5 em multinodular yellow-tan mass was adherent to the serosal surface of a

resected segment of bowel.

SPECIAL STAINS: Chromogranin: diffusely positive

CONTRIBUTOR: Mark Janssen, M.D. CASE NO. 4 - MAY 1997 Anaheim, CA

TISSUE FROM: Left groin mass ACCESSION #28018

CLTNICAL ABSTRACT: This35-year-old Asian-American male presented with a 2-3 year history of a mass in the left

groin area. Physical examination showed a 3.0 x 1.0 em subcutaneous hard lump in the groin. This was excised.

GROSS PATHOLOGY: A 5.2 x 2.5 cr:n ellipse of skin, and cut to a depth of 2.0 em, had a central, slightly raised area 3.5

x 2.4 em in diameter.

SPECIAL STAINS: CD34: Actin: S-100 protein:

negative negative positive

CONTRIBUTOR: David Huebner, M.D. CASE NO. S • APRIL 1997 Fontana, CA

TISSUE FROM: Abdominal wall ACCESSION #28032

CLINICAL ABSTRACT: Ten years ago this 76-year-old female had a total abdominal hysterectomy and bilateral salpingo­

·oophorectomy for stage 1, grade II endometrial carcinoma, followed postoperatively with adjuvant radiation. Two years ago she presented with a 3 x 4 em mass in the abdominal wall. This was resected and she was lost to follow-up. She.now presents with an enlarging 6.0 x 9.0 x 15.0 em abdominal wall mass. The mass was again resected.

GROSS PATHOLOGY: The specimen consisted of a.28.0 x 7.0 em ellipse of skin, with underlying fibroadiP.Ose tissue

and skeletal muscle. Within the muscle was a 23.0 x 11.0 x 7.6 em relatively well-circumscribed, lobulated, rubbery, firm pink-tan mass.

SPECIAL STAINS: Keratin cocktail, low molecular weight keratin (CAM 5.2), B-72.3: positive EMA, Vimentin: focal positivity Leu-M1: focal luminal positivity

(tumor cells essentially negative) CDA, AFP, SlOO, Chromogranin: negative

CONTRIBUTOR: Chisa Aoyama, M.D. CASE NO. 6 - MAY 1997 Sylmar, CA

TISSUE FROM: Abdomen ACCESSION #28026

CLINICAL ABSTRACT: This 39-year-old Caucasian female had childhood polycystic kidney disease with recurrent

nephrolithiasis and chronic pyelonephritis leading to a right nephrectomy at age 18. At that time she had a right oophorectomy and hysterectomy for ovarian carcinoma. The tumor pathology is not known but there is a possible history that the tumor secreted androgens. she now presents with left flank pain. Work-up found a large abdominal mass which was debulked.

GROSS PATHOLOGY: The 2.3 kg, 20.0 x 20.0 x 10.0 em specimen consisted of multiple fragmented pieces of white­

tan tissue with focally hemorrhagic and myxomatous change.

SPECIAL STAINS: Vimentin: EMA: Desmin:

positive positive negative

CONTRIBUfOR: Charles C. Osborn, M.D. CASE NO. 7 - MAY 1997 Glendale, CA

TISSUE FROM: Uterus ACCESSION #27962

CLINICAL ABSTRACT: This 50-year-old female was found to have an enlarged uterus and a hysterectomy was

·performed.

GROSS PATHOLOGY: Within the uterus was an 11.0 em diameter myometrial mass. It was very edematous,

hemorrhagic and friable and appeared to be well circumscribed.

SPECIAL STAINS: CD-34: Desmin:

negative positive

CONTRIBUTOR: Dhnrnm M. Ramnani, M.D. Dallas, TX

TISSUE FROM: Spleen

CLINICAL ABSTRACT:

CASE NO. 8 - MAY 1997

ACCESSION #28193

This 13-year-old Caucasian male has had a progressively worsening splenomegaly, which had been symptomatic, causing some respiratory embarrassment and anorexia. ACT scan of the abdomen revealed compression of the pancreas and stomach. A subtotal splenectomy was performed.

GROSS PATHOLOGY: The 1533 grams portion of spleen was 22.0 x 9.5 x 7.5 em. The specimen was intact except for

an 11.0 x 10.0 em area close to the hilum, and the inferior pole where parenchyma was exposed. The specimen was serially sectioned in 1.0 em thick slides revealing firm, pale, red-brown parenchyma. No focal lesions were identified.

SPECIAL STUDY: Electron microscopy showed the splenic macrophages to be distended by well-defined

cytoplasmic lysosomes containing small irregular whorls of lamellar material consistent with sphingomyelin.

CONTRIBUTOR: Thomos Heinz, M.D. CASE NO. 9 - MAY 1997 Oronge, CA

TISSUE FROM: Liver ACCESSION #28105

CLINICAL ABSTRACf: This 9-month-old Caucasian female presented with vomiting, mainly at night. Physical

.examination revealed a large abdominal mass. Further workup showed an elevated alpha fetoprotein of around 30,000. Ultrasound, chest cr, MRI, and hepatic angiography demonstrated a very large right hepatic tumor which displaced what appeared to be the right portal vein into the left lobe of the liver. An extended right hepatic lobectomy was performed.

GROSS PATHOLOGY: The specimen consisted of a 12.5 x 10.0 x 8.0 em, 427 gram portion of liver with variegated

pale tan to red-tan glistening capsule. Within the liver was a 9.5 x 85 x 7.5 em, well-circumscribed variegated pale tan to gray-tan mass.

CONTRIBUTOR: F. Azlzl, M.D. CASE NO. 10 - MAY 1997 Fontana, CA

TISSUE FROM: Right thigh ACCESSION #28085

CLINICAL ABSTRACf: This 21-year old female presented with a right thigh mass measuring about 20 em, which had

been growing slowly for approximately two years.

GROSS PATHOLOGY: Four pieces of soft gray-pink, friable tissue, varied from 2.0 to 2.5 em in greatest diameter.

SPECIAL STAINS: Cytokeratin: EMA: S-100:

positive positive negative

CAUFORNl<\ TUMOR TISSUE REGISTRY

SUGGESTED READING (General Topics f rom Recent Literature): Relationship BetwCCJJ Pelvic Jym1Jh Node I.nvolvement and Other Disease Sites in Patients with

Ovarian Cancer. Gynecol Ouco/1997; 65: 164-168. Corrclntlon of Body Fat Distribution with Grade of Endometrial Cancer. Douchl T, ljuin B,

Nal<amura S, Oki T, Maruta I<, et al Gyn~U:ol Onco/1997; 65: 138-14-2. Cervical Cancer Staging Systems. The Endless Debate (Editorial). Bened ct JL Gynecol Onco/ 1991;

65: 6-7. Critical Analysis of the Current American Joint Committee on Cancer Staging System for Cutaneous

Melanoma and Proposal of a New Staging S)'stem. Buzaid AC, Rou MI, Bakb CM, Soong SJ, et al J ofOin Onco/ 1997; 15(3): 1039-1051.

Reappraisal of the Role of Axillary Lymph Node Diuection in the Conservative Treatment of Breast Cancer. J ofClin Onco/1997; 15(2): 691-700.

Kolloeytic Atypia in Papanicolaou Smears: Reproducibility and BiOilSy Correlations. Lee KR, Minter U , et al. Cancer (Cancer C}1opathoQ 1991; 81(February 25): 10-15.

Well-Differentiated Liposarcoma of the Retroperitoneum: A CUnlcOIJathologic Analysis of 20 Cases, with Particular Attention to the Extent of Low-Grade Dedifferentiation. Elgar F, Goldblum JR. Mod Potho/1997; 10 (February): 113-120.

Rlsk of Malignancy and Death in Neurofibromatosis. Poyhoncn M, Niemela S, et aL Arch PathollAh M ed 1991; 121 (February): 139-143.

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) 824-4788 FAX: (909) 478-4188

CASE NO. 1, ACCESSION NO. 28031 !11AY1997

INLAND CR!\IERS!DEISAN BERNARDINO> • Mc~J~Static adenocarcinoma, NOS (?medullary carcinoma of thyroid) ( I); Medullary thyroid carcinoma (I); Hunhle cell can:inoma (I); Papillary carcinoma or th)'lroid with a solid component (I). Orlcoe)'tic carcinoma (1), Undifferentiated carcinoma, thyroid (1).

~~~~~~~~~[~-Giant c:cli carcinoma or thyroid. carcinoma (1); Poorly differentiated carcinoma (1). • Poorly differentiated (insular) carcinoma, thyroid (6). adenocarcinoma (5).

~~~~~~~~~~~~~ • Undifferentiated (anaplastic, sarcomatoid) careinoma of thyroid. - Anaplastic CA, NOS (7); Anaplastic CA, arising from insular CA (4); Hurthle cell CA (1).

~~;A;~&!JAE~(Ql~~~l!illill · Anaplastic carcinoma, thyroid. ~:§:lf~~~~:;~~~= · Poorly differentiated caicinoma ..,~~~tife:::Ja;~ carcinoma, r/o thyroid medullary carcinoma (2); Malignant

nec1p1asm, thyroid. medullaty carcinoma vs paraganglioma, malignant (1). BAX AREA • Medullary carcinoma of thyroid (anaplastic carcinoma developing from a follicular

carcinoma cannot be excluded) (3). SACRAMENTO <UC Davis> - Poorly differentiated thyroid carcinoma with Hurtble c:cli and anaplastic

features. ARIWNA ITucsonl • Poorly differentiated thyroid carcinoma [Insular carcinoma). WYOMING • Anaplastic thyroid carcinoma TEXAS ffil Paso) - Anaplastic carcinoma of thyroid (2). NEW MEXICO CAibuquerouel - Medullazy carcinoma with anaplastic features. NEBRASKA <Creighton University) - Thyroid carcinoma (Hunl~e cell vs papillazy variant). FLORIDA <Be1hcsda Naval Med Ctrl • Alveolar soft parts sarcoma. MARYLAND <Bethesda Naval Med Ctrl - Poorly dill'crentiated thyroid earcinoma (10). Anaplastic

thyroid carcinoma (4). NEW HAMPSHTRE (Manchester) - Adenocarcinoma with papillary Hurthle cell features (3). NEW JERSEY <Overlook Hospital Summill • Anaplastic thyroid earcinoma (1); Papillary thyroid

carcinoma (2). NEW YORK - Poorly differentiated thyroid carcinoma, insular type (thyroglobulin+) (4). MASSACHUSETTS <Berkshire Med Ctrl • Paraganglioma (2); vs medullaiy CA of thyroid (4). MAINE <Bangor) - Oncocytic carcinoma. CQNNECTJC!l[ - Anaplastic careinoma, thyroid. AUSTRALIA <Sydney> • Malignant paraganglioma JAPAN CShjrnada-KyotO) • MeduUazy carcinoma of thyroid.

DIAGNOSIS: POORLY DIFFERENTIATED TIIYROID CARCINOMA WITH HURTBLE CELL FEATURES

1'96000, M82903

NOTE: Inununostains for keratin and thyroglobulin were positive. Stains for chromogtanin, synaptophysln and S-100 protein \vere negative. Electron microscopy showed microacinus formation.

REEER£NCES: Watson RG, Brennan MD, Goellner JR. et al Invasive Hurthle Cell Can:inoma of the Th)roid Natural History and

Mllllllgemenl Mayc Cli11 Proc 1984; 59: 851-855. Gan:angiu ML, Bionchl S, Savino D, et at Follicular Hurthle Cell Neoplasms of the Th)'TOid Gland. A Study or IS3

C.S.. Cmct~r 1991;68: 1994-1953. Mueller-Oaertner HW, Bnac HT, Rebpemning W. Prognootic Indices for TIJmor Rclapse and Tumor Morlality in

Follicul.vThyroid Careinoma. Cancer 1991; 67; 1903-1911.

CASE NO. 2, ACCESSION NO. 28036 MAY 1997

INLAND <RIVERSIDE/SAN BERNARDINO> - Ossifying fibroma (3); Fibrous dysplasia (I), Desmoplastic fibroma (2).

CAMARILLO Wviso Group) - Non-ossifying fibroma. \IENTIJRA CUnilabl - Fibrous dysplasia (2). PLEASANTON/FREMONT - Desmoplastic fibroma vs fibrosarooma (6). LONG BEACH - Fibr<H>sseous lesion consistent with ossifying fibroma (5). QXNARD CSt. Johns Recional Medical Ctrl - Fibrous dysplasia/rio ossifYing fibroma. SAN DIEGO <Naval Me4 Ctrl - Benign fibr<H>SSeous lesion, most consistent with fibrous dysplasia (14). SANTA BARBARA CCouage Hosnita!l - Ossll)1ng fibroma. SANTA CLARA <Lorna Prieta Group) - Low grade ostcosarooma. SANTA ROSA - Fibrous dysplasia (I); r/o ossifying fibroma/non-ossifying fibroma, fibrous dysplasia (I);

Fibrous dysplasia r/o non-ossifying fibroma (1). BAY AREA - Fibrous dysplasia/benign fibro-osseous.tumor (3). SACRAMENTO cue Davis> - Fibro-osseous lesion. ARlZONA (f)Jcson l - Desmoplastic fibroma. WYOMING - Low grade fibrosarcoma of bone. TEXAS lEI Paso) - Fibrous dysplasia (2). NEW MEXICO CA!buoueroue) - Desmoid tumor. NEBRASKA CCreigluonl - Ossifying fibroma. FLORIDA CBcthesda Naval Med Ctrl - Fibrous dysplasia. MARYLAND CBethesda Naval Medical Center) - Ossifying fibroma (14). NEW HAMPSHIRE (Manchester) - Odontogenic fibroma vs fibrous dysplasia (I); Fibrous dysplasia (2). NEW )ERSEY <Overlook Hosoital Summit) - Desmoplastic fibroma (I); Ossifying fibroma (2). NEW YOR!( - Ossifying fibroma (4). MASSACHUSETIS <Berkshire Med Ctr) - Osteosarcoma (6) MATNE <Bangor) - Non-ossifying fibroma CONNECTICUT - Parosteal osteogenic sarcoma AUSTRALIA CSvdncyl - Ossifying fibroma JAPAN !Shi111<1dn-Kyotol - Desmoplastic fibroma.

DIAGNOSIS: FIBROMATOSIS, RIGHT MANDIBLE

TI0180, M76100

CONSULTATION: .•

Roben B. Brannon, MD., ChaiiiiUUl, Department of Oral Patl10log;y, AFIP, Washington, D. C. "Fibromatosis."

REFERENCES: Fu YS, Perz.in and Penin KH. NonepitheliBI Tumor of the Nasnl Cavity, Para-Nasal Sinuses and Nasopharynx. A

Clinicopathologic Study VI. Fibrous Tissue Tumors. Fil::rorna, Fibromatosis, Fi:bro5arcoma. Ccnctr 1976; 37: 2912 Takagi M, Yamamoto H, Mega H. etal. Hetetos=ity in the Gingival Fil::romatosis Canur 1991; 68: 2202-2212. Zain RB and Fct YJ. Fibrous Lesions of !he Gingiva. A Histopathologic Analysis of204 Cases. Oral Surg Oral Med,

Oral Patltol 1990; 70(4): 466-470. Oshiro U, Fukuda T, Tsuneyoshi M Fibrosarcoma Versus Fibromatosis and Cellular Nodular Fasciitis. A Coolparativc

Study of their Proliferative Activity Using Proliferating Cell Nuelcar Antigen, DNA Flow Cyt<lliielty, and pS3. Am J SurgPotholl994; 18(7): 7U-719.

FowterCB, eta.!: Fibromatosis oflheOrnl arul Parora1 Rqion. Oral Surg Oral Med Oral Palh, 1994; 77:373-386.

CASE NO. 3, ACCESSION NO. 28210 MAY1997

INLAND <RIVERSIDE/SAN BERNARDINO> • Melastatic pheochromocytoma (3); E.xtrn adrenal paraganglioma (1); Paraganglioma (2).

CAMARILLO (Alviso Grouol • Organ of Zuekerkandl catcinoma. VENTURA Nnilabl - Pheochromocytoma (2), PLEASANTQNIFREMONT • Paraganglioma (6).

LONG BEACH • Pheochromocytoma (5 ). QXNARD CSt Johns Regjonal Medjcal Ctrl • Pheochromocytoma (extra aclrcnal). SAN DIEGO <Navel Med Ctrl - Paraganglioma (14). SANTA BARBARA (Collage Hosoitall • Malignant paraganglioma SANTA CLARA CLomn PrieJa Group) • Malignant pheochromocytoma. SANTA ROSA - Paraganglioma (e.xtrn-adrenal pheochromocytoma XI); Paraganglioma (2). BAY AREA • Pbeochromocytoma (?men) (3).

SACRAMENTO rue Dayisl • Extra adrenal (malignant) Pheochromocytoma (3); Malignant paraganglioma (3).

ARIZONA Cf!!CSQnl • Pbeocbromocytoma. WYOMING • Neuroendocrine carcinoma. TE)(AS <EI Paso> - Pheochromocytoma (2). NEW MEXICO CAibu0uernuel - Carcinoid tumor. NEBRASKA (Creighton University) • Pheochromocytoma. FLORIDA <Bethesda Nayal Med Ctr) • Pheochromocytoma. MARYLAND CBe!besda Naval Me<! Ctrl - Pamgangliomalpbeochromocytoma, malignant (14). NEW HAMPSH1RE (Manches!er) • Pheochromocytoma, malignant(3). NEW JERSEY <Overlook Hospital Summit) • Malignant functional paraganglioma (I);

Pheochromocytoma, metastatic (2). NEW YORK • El\1rn·adrenal paraganglioma (4). MASSACHUSE'ITS <Berkshire Me<! Ctrl • Paraganglioma MAINE - Paraganglioma, malignant. CONNECTICUT - Paraganglioma AUSTRALIA <Sydney) - Extra adrenal pheochromocytoma. JAPAN CShimada-Kyotol • Paraganglioma.

DIAGNOSIS: 1\-lALlGNANT PARAGANGUOMA, PEL VIS

T¥6000, M86801

Note: Not all study set slides showed vascular involvement or vaswlar invasion.

FOLLOW-UP: A month later the patient expired. No autopsy was perfonncd.

REFER£NCES: TIUnors of the Extro-Adrenllll'araganglion System (Including Chemoreceptors). AtlaJ o/Tumor Pathol, S..:Ond Series,

Fascicle 9, AFTP. Washington, DC. 38-194. Linnoi.la RJ; Keiser HR, Steinberg SM and Lack EE. Histopathology of Benign Versus Malignon! Sympathetic Adrmal

P0t11gangliomas. A Clinieopatholo&ic Study of 120 Cases, Including Unusual Histologic Features. Hum Patloo/21: 1168-11 &0.

' CASE NO. 4, ACCESSION NO. 28018 MAY1997

lNLAND <RIVERSIDE/SAN BERNARDINO\ - Granular cell tumor (5); Granular cell myoblastoma (1). CAMARTI.-LO <Alviso Grouo) - Granular cell myoblastoma. VENTIJRA <Unilabl - Granular cell tumor (2). PLEASANTON/FREMONT - Granular cell tumor (6).

LONG BEACH - Granular cell tumor (5). OXNARD (St. Johns Regional Medical Ctrl - Granular cell tumor (granular cell myoblastoma). SAN DIEGO <Naval Med Ctr\ - Granular cell tumor (15).

SANTA BARBARA (Collage Hosoitall - Granular cell tumor. SANTA CLARA CLoma Prieta Group) - Granular cell tumor. SANTA ROSA - Granular cell tumor (3).

BAY AREA - Granular eel! tumor (3).

SACRAMEN'I'O IUC Davis) - Granular cell tumor (with atypical histologic features). ARIZONA crucsonl - Granular cell myoblastoma. WYOMING - Granular cell tumor. TEXAS CEI Paso\ - Granular cell tumor (2). NEW MEXICO (Albuquerque) - Granular cell tumor. NEBRASKA (Creighton University) - Granular cell tumor. FLORIDA CBethesda Naval Mcd.CJr) - Granular cell tumor. MARYLAND (Bethesda Naval Medical Center) - Granular cell tumor (14).

NEW HAMPSHIRE (Manchester\ - Granular cell tumor(!); Granular cell myoblastoma (2). NEW JERSEY (Overlook Hospital Summit) - Granular cell tumor (3).

NEW YORK - Granular cell tumor (4).

MASSACHUSEITS CBerkshire Med Ctr\ - Granular cell tumor. MAINE CBangorl - Granular cell lumor. CONNECTICUT - Granularcell .tumor. AUSTRALIA CSydney) - Granular cell tumor. JAPAN CShimada-Kyoto) - GranuL1r cell twnor.

DIAGNOSIS: GRANULAR CELL TUMOR, LEFT GROIN

TY7000, M95800

REFERENCES: Le Boil PE. Barr RJ, Burall S, Metcalf JS, et al. Primitive Polyposis Gnmular-€ell Twnor and Other Cutaneous Granular

Cell Neoplasms o(ApparentNonnewal Origin. AmJ Suf'8 Potho/1991; 15(1): 4~58. Lee J, Bhawan J, W~x F and Farber J. P.lexifonn Granular Cell Twnor. A report ·of Two Cases. Am J Dtmriztopadu>l

1994; 16(5): 537-54 I. Bakos L. Multiple Cutaneous Gnmular Cell Tumors with Systemic Defects. A Distinct Entity? lnt J D.!m~aUJ/1993;

32(6): 432-435. Patnaik AK. Histologic and Immunohistochemical Studies of Granular Cell Tumors in Seven Dogs, Three Cats, One

Horse, and One Bird Vet Parho/1 993; 30(2): 176-185. Kurtin PJ and Bonin DM Immunohistochemical Demonstmtion of the Lysosome-Associated Glycoprotein CD68 (KP·I)

in Granular Cell Iumors·and Sehwannomas (See Conunents). Hum Patho/1994; 25(11): 1172·1178. Sayer HP, Metze D, and Keel H. 'Granular Cell Dcnnatofibroma. AmJSurg Patholl991; 19(2): 168-173.

CASE NO. S, ACCESSION NO. 28032 MAY 1997

INLAND CRMRSIDEISAN BERNARDINO> - MetaStatic adenocarcinoma clw endomelrial origin (2); Carcinosarcoma (malignant mixed mullerian tumor) (I); Mewaatic: c:arcinoma (1); Poorly diffcrentiat.ed adenoe31cinoma perhaps from previous malignancy (clear cell endometrial) (2).

CAMARILLO (Alviso Group) - Adenoac:anlhoma. VENTIJRA <Unilabl - Poorly differentiated carcinoma (2). PLEASANION/FREMONT -Recurrent endomelrial carcinoma, high grade (4); Poorly differentiated

carcinoma witll clear cell areas (2). LONG BEACH - Carcinoma, NOS (5). QXNARQ <St. Johns Regional Medical Ctrl - Metastatic clear cell carcinoma of endometrium. SAN DIEGO <Naval Mcd Ctr\ • Metasiatic poorly-differentiated adenocarcinoma (3); Metastatic clear cell

adenocarcinoma (9). SANTA BARBARA (Cottage Hosoitall - Carcinosarcoma. SANTA CLARA <Lorna Prieta Group) - Oear·cell carcinoma. SANIA RQSA - Metastatic carcinoma, probably from endometrial clear ceD carcinoma ( I ); Poorly

diffcrentiated epilhelial malignancy carcinoma ( I); Poorly differentiated carcinoma (I). BAY ABEA - Adenoe31cinoma, c:lear cell variant metaSt.atic (3). SACRAMENTO rue Davis\ - Metastatic adenocarcinoma. ARIZONA ffitcson\ - AdenOC31Cinoma. WYOMING - Moderately differentiated metastatic adenoe31cinoma. TEXAS lEI Paso> - Metastatic adenocarcinoma (2). NEW MEXICO CAlbuqueroue) - Poorly differentiated carcinoma, probably adenocarcinoma. NEBRASKA CCrej@ton University) - Adenocarcinoma (metastatic vs impla.nted). FLORIDA <Bethesda Naval Med Ctr\ - Recurrent endometrial carcinoma. MARYLAND <Bethesda Naval Medical Center\ • Metastatic adenocarcinoma (14). NEW HAMPSHIRE <Manchester) • Metastatic adenocarcinoma (1); Metastatic poody differentiated

carcinoma (2).

NEW JEBSEY <Overlook Hosoital Swnmitl - Clear cell adenocarcinoma (I); Adenocarcinoma poorly­differentiated (NOS) (2).

NEW YORK - Metastatic: adenocarcinoma will! clear cell features, most likely of mullerian origin, in particular, from llle patient's known endometrial primary (4).

MASSACHVSETI$ (!lerkshire Med Ctrl - Carcinosarcoma. MAINE @an cor) - Metastatic adenocarcinoma. CONNECDCUT • Ocar cell carcinoma, differential diagnosis mesolllelioma. AUSTRALIA <Sydney) - Clear cell carcinoma (metastatic). JAPAN CShjQ)I!da-Kyo!ol - Synovial sarcoma .

.DIAGNOSIS: POORLY DIFFERENTIA TED ADENOCARCINOMA WITH CLEAR CELL FEATURES, PlWBABLY OFMUL:LERIAN ORIGIN, ABDOMlNAL WALL

Il(4300,M83103.

FOLLOW-UP: The patient had a recurrence six months post-operatively, whicb was nxxcised.

REFERENCES; None.

CASE NO. 6, ACCESSION NO. 28026 MAY 19'>7

INLAND CRIVERSIDEISAN BERNARDINO> - Ncplvoblastoma (2); Malignant neuroectodermal tumor ( I); Adult Wilms tumor (I); Endodennal sinus tumor (I); Undifferentiated crucinoma (I).

CAMARILLO <Alviso Grouol - Granulosa cell carcinoma. VENTURA <Unilabl - Granulosa cell tumor (2). PLEASANTQNIFREMONT - Sertoli Leydig cell tumor, grade ill (sarcomatoid) (6). LONG BEACH - Carcinosarcoma (5).

OXNARD <St. Johns Regional Mcd Ctrl - SertoU-Leydig cell tumor, intermediate (Meyer's 11) sex cord tumor/rio granulosa cell tumor.

SAN DIEGO <Naval Med Ctrl - Endomclrioid CA with sex cord stroma features (7); Se>< cord stromal twnor (5); Granulosa celltwnor (3).

SANTA CLARA <Loma Prieta Group> - Juvenile granulosa cell tumor. SANTA ROSA - Poorly differentiated Sertoli-Leydig celltwnor (I); Gyandroblastoma, residual, recurrent

by blstory (I); Sertoli Leydig cell neoplasm(!). • BAY AREA - Malignant sex cord stromal tumor (2); Carcinosarcoma (I). SACRAMENTO CUC Davis> - lmmatll!C teratoma. AR!ZONA Cfucsonl - Low grade malignant teratoma. WYOMING - Poorly differentiated Sertoli-Leydig cell tumor- metaslatic. TEXAS CEI I'll so) - SertoU-Leydig oell tumor, poorly differentiated (2). NEW MEXICO (Aibuauerauel - Sertoli-Leydig cell tumor intermediate differentiation. NEBRASKA <Creighton University) - Immature teratoma. FLORIDA <Bethesda Naval Med Ctrl - Granular cell tumor. MARYLAND <Bethesda Naval Medical Center) - Sertoli-Leydig cell tumor, retiform variant (10);

Scrtoliform variant or endometrioid carcinoma (2); Small cell carcinoma (2).

NEW HAMPSHIRE (Manchester) - Malignant Brenner's tumor (I); Favor Sertoli-Leydig cell tumor (intermediate -poorly differentiated) (2).

NEW YORK - Sertoli-Leydig cell tumor (androblastoma) (4).

MASSACHUSETIS <Berkshire Med Ctrl - Sertoli celltwnor. MAINE <Bangor> - ? Sertoli cell vs immatll!C teratoma. CONNECDCVT - Sertoli-Leydig cell tumor of intermediate grade. AU§TRAL!A <Sydney) - Sertoli-Leydig cell twnour (poorly differentiated). JAPAN <Shimada-Kyotol - Sex cord- stromaltwnor.

DIAGNOSIS: SERTOLl-LEYDIG TUMOR, ABDOMEN

TY4100, M86JIO

CONSULTATioN: Dr. Fu from UCLA foels that the differential diagnosis should include "Immature Teratoma and

Sertoli-Leydig Cell Tumor."

REfERENCES:

Young RH and Seully RE. Ovarian Sertoli-Leydig Cell Tumon. A Clinic:opelhological Analysis of207 Cases. Am J Surg Patlroll985; 9(8): S4l-569.

CASE NO. 7, ACCESSION NO. 27962 MAY1997

INLAND CRIVEBSrDEISAN BERNARDINO> - Endomclrial stromal nodu«: (I); Stromal nodule; Cellular leiomyoma (I); Vascular leiomyoma (I); Epithelioid leiomyoma, plexiform type (I); Angiomatosis (I).

CAMARILLO (Alviso Grouol - Angiosarcoma VE!:{ruRA CUnilabl - Stromal nodule (1); Endometrial stromal nodule vs low grade stromal sarcoma (1). PLEASANTON/FREMONT - Leiomyoma, cellular and plexifonn (4); Stromal nodule (2).

LONG BEACH - Lymphangio-lelomyoma (3); Pericytoma (2).

OXNARD <St. Jolm 's Regional Med Ctrl - Endometrial stromal nodule vs low grade stromal carcinoma. SAN DIEGO fNnyal Med Ctrl - Low grade stromal sarcoma (12); Endometrial stroma) nodule (3).

SANTA BARBARA <Cottage Hospjtal) - Endometrial stromal nodule. SANTA CLARA CLoma Prieta Grouol - Endometrial stromal san:oma. SANTA ROSA - Low grade endometrial slromal sarcoma (1); Cellular leiomyoma, mitotieally inactive

(atypiea1)(1); Vascular leiomyoma vs stromal sarcoma (I).

BAY AREA - Endometrial stromal tumor (2); Mixed leiomyoma and endometrial stromal nodule (1). SACRAMENTO I'Dlcson> - Endometrial stromal nodule. ARIZONA (Aibugucmue) - Vascular leiomyoma. wyOMING - Low grade endometrial slromal sarcoma. TEXAS CEI Paso) - Endometrial stromal sarcoma, low grade (2).

NEW MEXICO - Endometrial slromal sarcoma, low grade. NEBRASKA <Creighton University) - Large stromal nodule (1); Low grade stromal sarcoma (I). FLORIDA <Betbesda Naya! Med Ctrl - Vascular leiomyoma. MARYLAND <Bethesda Naval Medical Center\ - Endometrial stromal tumor (13); Leiomyoma (1).

NEW HAMPSHlRE (Mancltester) - Atypiealleiomyoma with degenerative features (I); Endometrial stromai nodule (2).

NEW JERSEY <Overlook Hosoital Symmi!) - Benign endometrial stromal tumor (3).

NEW YORK - Epithelioid (plexifonn) leiomyoma of uncenain malignant pol~ntial (4). MASSACHUSE'IT$ (Berkshire M¢ Qrl - Endometrial stromal tumor. MAINE CBangoO - Cellular leiomyoma with prominent vascularity. CONNECTICUT • Slromal sarcoma, differential diagnosis ofHemangiopericytoma. AUSTRALIA <Sydnevl • Endomelrial stromal nodule. JAfAN <Shimada·Kvoto) - Epilbelioid leiomyosarcoma.

DrAGNOSIS: ENDOMETRIAL STROMAL NEOPLASM, PROBABLY STROMAL NODULE

T8400Q, M8000l

CONSUL TA TIQN: Richard Kempson, MD., Stanford University Health Center: Endometrial stromal neoplasm,

probably stromal nodule (based mostly upon its circumsaiption).

FOLLOW-UP: No recurrence of the tumor after one year.

REFERENCE$: Chang KL, Crabtree GS, Lim-Tan SK, d aL Primary Uterine Endometrial Stromal Neoplasms. A Clinicopathologic

Studyofli7Cascs. AmJSurgPathoJ 1990; 14(5): 4JS-438. Kempson RL and Hendrickson MR. Pure Mesenchymal Neoplllsm of the Uterine Co!pus. Selected Problems. Semin

Diag PatltoJ 1988; 5(2): 172-~98.

CASE NO. 8, ACCESSION NO. 28193 MAY1997

INLAND CR! VERSIDEISAN BERNARDINO> • Niemann-Pick disease {5); Lipid storage disease (Histology not characteristic of citl1er Gaucltcr or Niemann Pick).

CAMARILLO CAiviso Grouol • Histiocytosis X. VENTURA aJnilabl - NiemaM Pick (I); Lipid histiocytosis c/w Niemann Pick disease (I). PLEASANTQNIFREMONT • Sphingomyelin Lipidosis (Niemann-Pick disease) (6). LONG BEACH - Sphingomyelin lipid storage disease (5).

OXNARD CSt Johns Reruonal Medical Ctrl - Lipid storage histiocytosis c/w Niemann Pick disease. SAN DIEGO CNaval Me4 Ctrl • Niemann-Pick disease, type B (15). SANTA BARBARA (Cottage Hospital) • Niemarut Pick disease. SANTA CLARA (Lorna Prieta Group) • Niemann-Pick disease, type B. SANTA ROSA • Niemarut-Pick disease (2); Sphingomyelin-lipidosis (Niemarut-Pick) (I) BAY AREA - Ncimann·Pick disease (3); (?Fabry's). • SACRAMENTO aJC Davis> - Niemann Pick disease, type B. ARJmNA Cfucsonl • Nicmarut·Pick disease, benign variety. WYOMING • Niemarut Pick disease. TEXAS CEI Pasol • Niemarut Pick disease (2). NEW MEXICO CAibuguerauel - Niemann Pick disease. NEBRASKA <Creighton UnivcrsiM - Ceroid histiocytosis (NiemaM Pick. type B). FLORIDA CBethesda Nayat Med Ctrl • Niemann Pick. MARYLANQ CBethesda Nayal Medical Center> • Sphingolipidosis (Niemann Pick disease (14).

NEW HAMPSHIRE CMancheslerl - Niemarut Pick disease. NEW JERSEY COyerlook Hosoj!a! Swnmitl - NicmaM Pick disease (3). NEW YORK • Niemaru1-Pick disease (4).

MASSAC!fUSETIS (Berkshire Med Ctrl - Niemann Pick disease, type B. MAINE CBangorl • Niemann Pick disease. CONNECTIC\IT • Gaucher's disease. AUSTRALIA CSydnevl • Niemann-pick disease. JAPAN CShimada·Kvotol - Niemann pick disease.

DIAGNOSIS: NIEMANN-PICK DISEASE, SPLEEN

T07000, DI420

FOLLOW-UP: A year an.d a half after surgery the patient is asymptomatic and doing weU.

REFERENCES: Dawson PJ Md Dawson CT. Adult Niemann-Picl: Disease with Sea-Blue Histioeytes in the Spleen. Hum Patho/1982;

13: 1115-1 \20. SilversteinMN, Ellefson RD,and Ahem ET. The Syndrome of the Sea-blue Histiocyte. N EngJMcd 1970; 282: 1-4. Brunning RD. Morphologic Allerations in Nucleated Blood and Marrow Cells in Genetic Disorda's. Hum Patho/!910;

1: 99-124. Brady RO. Sphinomyelin Lipidooes. Niemann-Picl: Disease. In Stanbury JB, Wyngaarden JB, Fric:kricl:soo DS,

Goldstein JL, Brown MS {Ed$). Tite Me!Abolicllasis of Inherited Disease. New York 1983, McGraw-Hill Book Company: 831-841.

CASE NO. 9, ACCESSION NO. 28105 MAY 1997

INLAND !RIVERSIDE/SAN BERNARDINO> - Hepatoblastoma, favorable fetal pattern (I); Hepatoblastoma (4), Adenoma (I) ..

CAMARILLO <Alviso Group) - Juvenile hepatocellular carcinoma. VENTURA <Unilabl - Hepatoblastoma (2).

PLEASANTON/FREMONT - Hepatocellular carcinoma (5); Hepatoblastoma (1).

LONG BEACH - Childl1ood hepatocellular carcinoma (5).

OXNARD <St. Johns Regional Med Ctrl - Hepatoblastoma. SAN OrE GO <Nayal Med Ctrl - Epithelial hepatoblastoma (15). SANTA BARBARA <Cottage Hospital) - Hepatoblastoma. SANTA CLARA fLoma Prieta Groupl - Hepatoblastoma SANTA ROSA - Hepaioblastoma, epithelial type (1); Hepatocellular carcinoma, r/o adenoma(!);

Hepatoblastoma (1).

BAY AREA - Hepatoblastoma with EMH (3).

SACRAMENTO <UC Davis) • Hepatoblastoma (fetal type).

ARIZONA (Tucson) - Hepatoblastoma. WYOMING - Hepatoblastoma. TEXAS <El Paso) - Hepatoblastoma (2).

NEW MEXICO (Albuquerque) - Hepatoblastoma. NEBRASKA <Creighton Universitvl - Hepatoblastoma (1); Hepatocellular carcinoma(!). FLORIDA <Bethesda Naval Med Ctr) - Hepatoblastoma. MARYLAND <Bethesda Naval Medical Center> - Hepatoblastoma, epithelial type (14).

NEW HAMPSHJRIHManchesterl - Hepatocellular carcinoma (3). NEW JERSEY <Overlook Hospital Summit) - Hepatoblastoma (3). NEW YORK - Hcpatoblastoma (4).

MASSACHUSE1JS <Berkshire Med Ctrl • Hepatoblastoma, fetal type.

MAINE <Bangor) - Hepatoblastoma. CONNECriCIJf - Hepatoblastoma. AUSTRALIA <Sydney) - Hepatoblastoma. JAPAN <Shimada-Kvotol - Hepatoblastoma.

DIAGNOSIS: HEPATOBLASTOMA

T5600.0, 1\<189703

REFERENCES: Conran RM, Hitchcock CL, Maclawi W, et 81. Hepatoblastonia. The Prognostic Significance ofHistologic Type.

Pediacarpatho/1992; 12: 167-183. Go112ales-Crussi F, Upton MP and Mauer HS. Hepotoblastoma. Attempt at Characterization of Histologic Subtypes. Am

J Surg P~tho/1982; 599.§12. Lacke E, Ncave C and Vawter GF. Hepotoblastoma. A Clinical and Pathologic Sltldy of 54 Cases. Am J Surg Pathol

1982; 6: 693-705. Saxena R. Leake, JL, Shafford EA, Thwenport M. et al. Chemotllempy Effects on Hepotoblastoma. AmJ Surg Pathol

.1993; 17(12): 1266-1271.

CASE NO. 10, ACCESSION NO. 28085 MAY 1997

!NLANQ <RIVERSIDE/SAN BERNARPINO> • Monophasic, synovial sarcoma, fibrous type (2); Synovial sarcoma (I); Epithelioid sarcoma (I); Epithelial sarcoma-fibrosarcoma pattern (I); Malignant schwannoma (I).

CAMARILLO <Alviso Groupl - Schwannoma yENTURA <Unilab) - Synovial sarcoma (2). PLEASANTON/FREMONT - Synovial sarcoma (5); Leiomyosarcoma (I). LONG BEACH - Synovial sarcoma (5). OXNARD CSt. Johns Regional Med Ctrl - c/w synovial sarcoma. SAN DIEGO <Naval Med CCtrl - Synovial sarcoma, monophasic (IS). SANIA BARBARA <Cottage Hosoj(al) - Synovial sarcoma. SANIA CLARA <Lorna Pric(a Group) - Monophasic synovial sarcoma. SANTA ROSA - Synovial sarcoma (I);? mesenchymal neoplasm, r/o synovial sarcoma (I); Mesenchymal

neoplasm vs epithelial neoplasm.undilferentiated, r/o synovial sarcoma (1). BAY AREA - Syno\~al sarcoma, monophasic (3) .. SACRAMeNTO <UC Davis) - Synovial sarcotruL ARIZONA ffi1csonl - Monophasic synovial sarcoma. WXOMJNG • Monophasic synovial sarcoma. TEXAS lEI Pasol - Synovial sarcoma (2). NEW MEX!CO CAibJJ91Jemuel - Malignant neoplasm, favor sarcoma, possible synovial sarcoma,

monophasic. NE8RASKA <Creighton University) - Monophasic. FLORIDA <Bethesda NaYj!l Mcd C!rl - Monophasic synovial sarcoma. MARXLAND <Bethesda Nayal Mcd Ctrl - Synovial sarcoma (14). NEW HAMPSHIRE <Manchester) - Synovial sarcoma (3). NEW JERSEY (Overlook HosniJal Summit) - Synovial sarcoma. monophasic type (3). NEW YORK - Synovial sarcoma (4). MASSACHUSETIS <Berkshire Mcd Ctrl • Synovial sarcoma. MAINE <Bangor) - Synovial sarcoma. CONNECTICUT - Synovial sarcoma. AUSTRALIA CSvdnevl - Synovial sarcoma. JAPAN CShirnada-Kvotol - Synovial sarcoma.

DIAGNOSIS: MONOPHASIC SYNOVIAL SARCOMA, RIGHT TIDGH

T¥9100, M90403

CONSULTATION: Christopher D. M Fletcher, M.D. Harvard Mcdieal School. "Monophasic Synovial Sarcoma."

REfERENCES: Oda Y, Hashamoto H. Takeshita Sand TS>mcyoshl M. The Prognostic Value oflnununOOi.stocl!emical Slaining for

Pro1ifemling Cell Nuclear Antigen (PCNA) in Synovial San:oma. Ct:ncu 1993; 72: 47&-4&5. Lopes 1M, Bjeri;ellagen B, Holm R, ctal. Immunohistochemical Profile of Synovial Sarcoma with Emphasis on the

Epithelial-Type Differentiation. A Study of 49 Primal)~ Tumcn, Recurrences and Metastases. Parhol Ru Pracr 1994; 190: 168-177.

MacKenzie DH. Monophasic Synovial Sllrcoma. A Histological Entity? HIJtoparho/1977; 1: 1S1-157. Miel!ulcn M. Keratin Subsets in Spindle Cell Sarcomas. Keratins are Widespread but Synovial Sarcoma Contains

Distinctive KC!lltin Polypeptide Pauern mld Desmoplakins. Am J Parho/1991; 138: 505-S13. Qda Y, Hashamoto H, TSUileyoshi M ruld Takcshila S. SUIVival in Synovial Sarcoma. A Multi variant Study of

Prognostic Factors mth Special Emphasis on Comparison Bctwocn Early Death and Lo03-T enn Sum val. Am J Surg Potho/1993; 17: 3544.