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CALIFORNIA TUMOR TISSUE REGISTRY

"GYNECOLOGIC PATHOLOGY" Study Cases, Subscription A September 1998

California Tumor Tissue Registry do: Department of Pathology aod Human Anatomy Loma L.inda University School ofMedicine 11021 Campus Avenue, AH 335 Loma Linda, California 92350 (909) 824-4788 FAX: (909) 478-4188 E-mail: [email protected] Target audience: Practicing pathologists and pathology residents.

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

Objective: The participant will be able to recognize morphologic features ofa variety of benign and malignant 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 ofselected references from the medical literature.

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

CMECredit: The CTfR designates this activity for up to 2 hours of continuing medical education. Participants must return their diagnoses to the CTTR as documentation ofparticip ation in this activity.

Accreditation: The California Tumor Tissue Registry is accredited by the California Medical Association as a provider ofcontinuing medical education. • CONTRIBUTOR: J. R. Craig, M.D. CASE NO. 1 • SEPTEMBER 1998. Pasadena, CA

TISSUE FROM: Right and Left ACCESSION #22287

CLINICAL ABSTRACT: This 26-year-old, GO PO, Caucasian female had a long history of endocrine problems, which included irregular and infrequent menses. She was obese and complained of hirsutism. Cervical and rectal exam proved negative. An exploratory laparotomy with bilateral ovarian wedge resection was performed.

GROSS PATHOLOGY: . The 23 gram wedge resection of cystic right was 9.0 x 3.0 x 2.0 em. It had multiple serous cysts up to 5 mm in diameter that occupied approximately 25% ofthe total mass. The 20 gram left ovarian wedge was 7.5 x 2.0 x 2.3 em and similar in appearance to the right ovary.

CONTRIDUTOR: Mark Janssen, M.D. CASE NO. 2 - SEPTEMBER 1998 Anaheim, CA

TISSUE FROM: ACCESSION #28322

CLINICAL ABSTRACT: This 57-year-old Caucasian female presented with a two-month history ofvaginal bleeding, which led to a D&C and then a hysterectomy.

GROSS PATHOLOGY: TQe 366 gram uterus was 11.5 em in length, 6.0 em from cornu to cornu and 5.0 em in the anteroposterior diameter. On the posterior aspect ofthe endometrial cavity was a 9.0 em diameter ulcerated, partially necrotic mass.

SPECIAL STUDIES: Keratin strongly positive • • CONTRIBUTOR: Peter L. Morris, M.D. CASE NO.3 - SEPTEMBER 1998 Santa Barbara, CA

TISSUE FROM: Ovary ACCESSION #28274

CLINICAL ABSTRACT: This 47-year-old, para 5, female bad a one year history ofp rolonged menstrual periods · associated with malaise, hot flashes, nervousness, illsomnia, weight loss, irritability, headaches, and anorexia. Cyclic hormones relieved most of her symptoms, but irregular menses continued. Pap smears were abnormal and continued to be after cryotherapy for proven cervical dysplasia. A vaginal hysterectomy and salpingo-oophorectomy were performed.

GROSS PATHOLOGY: The bilateral ovaries weighed 33 grams. One of the ovaries was enlarged to 4.2 x 3.0 x 2.5 em It was somewhat nodular and had a 1.0 em smooth-lined cyst and a I .2 x 1.5 em multiloculated cystic lesion. Within the larger cyst was a mixture of greasy yellow mate.rial and hair.

SPECIAL STUDIES: Chromogranin positive Thyroglobulin positive

CONTRIBUTOR: Peter L. Morris, M.D. CASE NO. 4 - SEPTEMBER 1998 Santa Barbara, CA

TISSUE FROM: Uterus ACCESSION #28194

CLINICAL ABSTRACT: This 50-year-old female presented with a three year history ofuterine leiomyomata and menorrhagia: Uterine size by palpation was around 14 weeks and in mid-position. Adnexa were not palpable. A total abdominal hysterectomy was performed.

GROSS PATHOLOGY: The enlarged uterus measured 14.0 x 11.0 x 8.0 em and weighed 645 grams. The endometrial cavity was distorted by multiple myometrial nodules which ranged from 1.2 to 5.5 em.

SPECIAL STUDIES: Keratin strongly positive in lesional cells ' CONTRIBUTOR: Cynthia L. Douglas, M.D. CASE NO. 5 - SEPTEMBER 1998 San Luis Obispo, CA

TISSUE FROM: Ovary ACCESSION #27640

CLINICAL ABSTRACT: This 84-year-old Caucasian female presented with shortness of breath and diaphoresis. She was found to have a urinary tract infection. Physical examination revealed a 20 em non-mobile lower abdominal mass.

GROSS PATHOLOGY: Specimen consisted ofa uterus with attached tube and ovary. The ovary was replaced by a 2425 gram tumor which measured 21.0 x 1-2.0 x 17.0 em. The parenchyma was solid fibrous, yellow­ gray with a central 4.2 em cyst.

CONTRIBUTOR: Boward Otto, M.D. CASE NO. 6 - SEPTEMBER 1998 Cheboygan, MI

TISSUE FROM: Left ovary ACCESSION #28203

CLINICAL ABSTRACT: This 54-year-old female presented with generalized abdominal pain and accompanying nausea. Examination revealed exquisite McBurney's tenderness, particularly to deep palpation. CT scan revealed two masses near the uterus which were cystic in quality. An appendectomy and bilateral oophorectomy were performed.

GROSS PATHOLOGY: The appendix showed acute appendicitis. The left ovary weighed 25 grams and consisted ofa 4.0 x 3.5 x 3.0 em nodule with a smooth glistening surface. Cut surface was solid, soft and gray with some yellow mottling. A small rim ofgray tissue in the cortex suggested Tesidual ovarian parenchyma. The tumor extended toward and into the capsule but not through the capsule. '

CONTRIBUTOR: Boward Otto, M.D. CASE NO. 7 - SEPTEMBER 1998 Cheboygan, MI

TISSUE FROM: Left ovary ACCESSION #28201

CLINICAL ABSTRACT: This 58-year-old female was found to have a left adnexal mass on routine examination. Ultrasound revealed a 4.0 em solid mass of the le.ft adnexa. A total abdominal hysterectomy with bilateral salpingo-oophorectomy was perfonned.

GROSS PATHOLOGY: The left ovary was replaced by a 50 gram, 6.0 x 5.0 x 4.0 em muhinodular cystic mass. Sectioning revealed a mottled tan multinodular suriilce that bulged above the cut surface ofthe ovary.

CONTRIBUTOR: Arno Roscher, M.D. CASE NO. 8 - SEPTEMBER '1998 Valencia, CA

TISSUE FROM: Both ovaries ACCESSION #28214

CLINICAL ABSTRACT: This 17-year-old female presented with amenorrhea. Gene studies showed a karyotype compatible with gonadal dysgenesis. It was recommended that both ovaries be removed due to a high incidence ofcancer. A bilateral oophorectomy was prefonned.

GROSS PATHOLOGY: The right ovary was submitted as two pieces offinn and fleshy, pink-tan tissue with areas of brown-black discoloration ofthe suriilce, each measuring approximately 2.0 x 2.0 x 1.5 em. The left ovary consisted ofa 3.0 x 1.5 x 1.5 em piece of firm and fleshy, pink-tan tissue with some dark brown discoloration ofthe surface. CONTRIBUTOR: Lorna Linda Pathology Group (kt) CASE NO. 9 - SEPTEMBER 1998 Lorna Linda, CA

TISSUE FROM: Uterus ACCESSION #28251

CLINICAL ABSTRACT: This 38-year-old female presented with a pelvic mass. An exploratory laparotomy with total · abdominal hysterectomy was perfonned.

GROSS PATHOLOGY: The 1375 gram, 19.0 x 17.0 x 10.0 em uterus with attached bilateral adnexa showed a firm white-tan mass which extended from the endomyometrium to the serosal surfuce. It also appeared to invade into the endocervical canal

SPECIAL STAINS: Desmin 3+ (of 4) Leukocyte common antigen negative Chromogranin negative Actin negative Keratin (cocktail and CAM 5.2) no significant activity S-100 focal faint positivity, but largely negative Synaptophysin no significant staining Glycogen no significant staining PAS weakly + in tumor cells

CONTRIBUTOR: D.M. Lawrence CASE NO. 10 - SEPTEMBER 1998 San Luis Obispo, CA

TISSUE FROM.: Left ovary ACCESSION #28240

CLINICAL ABSTRACT: This 88-year-old female presented with weight loss and weakness. Work-up revealed a 15 em left ovarian mass. The patient underwent a left salpingo-oophorectomy.

GROSS PATHOLOGY: The 501 gram, 15.0 x 10.5 x 7.2 em ovary had a solid and cystic parenchyma.

SPECIAL STAINS: Cytokeratin positive Vimentin positivity restricted to vascular structures Germ cell markers negative

C~e Studies, September 1998. Subscription A CALIFORNIA T UMOR T ISSUE REGISTRY

·-····. September 199!~, -~~

SUGGESTED READING (General Topics fr om Recent Literature):

E-Mail, lhc lmcrnet, and Information Access Technology in Palhology. Sem in Diag Patho/1994; 11{4): 294-304. DiGiorgio CJ, Cbarles A, Richert. etc. lnu:rpretatioo or the Literature. Clin Obstet and Gyn 1998: 41{2): 307-314. Dolan MS. Predicting Oinical Outcome for Uterine Smoolh Muscle Neoplasms wilh a Reasonable Degree of Certainty. lnt J Gynecol Patho/1995; 14: 243-249. Longacre TA. Hendrickson MR. and Kempson RL.

California Tumor Tissue Registry c/o: Deparnnent 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 E-mail: [email protected] CASE NO. 1, ACCESSION NO. 22287 SEPTEMBER 1998

INLAND (Riverside/San Bernardino) • Polycystic.ovary (2) BAKERSFIELD (San Joaquin Comm Hospi!all - Polycystic ovary disease (2). BAKERSFIELD CCen·traJ Valley Srudv Group) - Polycystic ovary. VF.. NTURA

DIAGNOSIS:

POLYCYSTIC OVARIAN DISEASE T87000, 02601

REFERENCES: Coney P. Polycytic Ovarian Disease. CWTent Concepts of Pathophysiology and Therapy. Fmil Steril J 984: 42:667- 6&2. Cooper HE, SpeUacy WN, Prom KA and Cohen WD. Hereditary Factors in the Stein-Leventhal Syndrome. Am J Obsrer Gyneccli96&: 100:371-387. Hugbesdon PE. Mo!phology and Mo!pbogenesis of tbe.Stein-Leventhal Ovary and So-Called ''Hyperthecosis", Obsrer Gynecol Surv 19&2; 37:59-77. Hutchison JR, Taylor HB, Zimmerman EA. Tbe Stein-Leventbal Syndrome and Coincident Ovarian Neoplasms. Obslel Gynecol 1966; 28:700,703. Dahlgren E.Johansson S, Lindstedt C, et aJ. Women with Polycystic Ovary Syndrome Wedge Resected in 1956 to !965. A Long T<'IlD Follow-Up Focusing on Natural History and Cin:ulating Hormones. Fertil Sreri/1992; 505- 513. Takahashi K, Eda Y, Abu-Mus a A. eta!. Transvaginal Ultrasound Imaging Histopathology and Endocrinopathy in Patients with Polycystic Syndrome. Hum011 Reprod·l994: 9:1231 ·1236. CASE NO. 2, ACCESSION NO. 28322 SEPTEMBER 1998

INLAND (Riverside/San Bernardino) - Clear cell endometrial carcinoma(!); Undifferentiated clear cell carcinoma (1). BAKERSFIELD

DIAGNOSIS:

HIGH GRADE PLEOMORPHIC LEIOMYOSARCOMA WITH LIPOSARCOMATOUS DIFFERENTIATION AND KERATIN EXPRESSlO!'I, UTERUS T82000,M88903

CONSULTATION: Mayo Clinic. "Poorly Differentiated Leiomyosarcoma with Liposarcomatous differentiation."

REfERf!NCI!S: Vakiani M, Mawad l. and Toletman A. Heterologous of the Uterus. /nt J Gynec<>l1982; 1:211 -219. Lundgren L, Kindblom LG, Seidal T, et al. Intermediate and Fine Cytofi!amenL< in Cutaneous and Subcutaneou• I..eiomyo.sarooma. APMIS {Denmark) 1991; 99{9):820·828. Miettinen M. Keratin Subsets in Spindle CeU Sarcomas. AmJ Patho/1991; 138(2):505-513. Miettinen M. Immunoreactivity for Cytokcratin and ·Epithelial Membrane Antigen in I..eiomyosill'coma. Arch Patho/ Lob Mcd 198&; 112(6):637-640. CASE NO. 3, ACCESSION NO. 28-274 SEPTEMBER 1998

INLAND

.DIAGNOSIS: STRUMAL CARCINOID TUMOR, OVARY T87000, M90911

REfEBENCES' Armes IE and Oster AGo A Case of Maligoan

INLAND

DIAGNOSIS:

ADENOMATOlD TUMOR OF UTERUS T820Q(), M90540

REFERENCES: Otis CN. Uterine Adenomatoid Tumots. Immunohistoebemical Characteristic.~! wiib Emphasis on Bc:·r.J!P4 Immunoreactivity and Distinction f'JOm . lnt J GyMcol Patho/1996; 15(2):146-151. FfO'enczy A. Fenaglio J and Richard RM. Observations on Benign of the Genital Tract (Adenomatoid Tumor). A Comparative Ultrasttuciurol Study. Cancer 1912; 30:244-260. Quigley JC ana Hart WR. Adenomatoid Tumors of theUterus. Am J C/in Pmlwl l 981; 76:627-635. Uvingston E. Guis M and Peart M. Diffuse Adenomatoid Tumor of U!erus with Serosal Papillary Cystic ComponenL tnt J Gynecol Pmho/ 1992; 11 :221!-292. CASE NO. 5, ACCESSION NO. 267

INLAND (Riverside/San Bernardino) - Cyst adenofibroma. ovary ( I); Atypically proliferating endomeuioid adenofibroma (1). BAKERSFIELD (San Joaquin Comm Hospital) - Adenofibroma (I); Metastatic adenocarcinoma to the ovary (!). BAKERSFIELD (Cenlral Valley Study Gmupl • Adenofibroma VENTURA CUnllabl - Adenofibroma (2). SANTA ROSA . Endomeuioid carcinoma (1); Malignan~ cndomeuioid carcinoma, rio Krukenbcrg \ i); Adenocarcinoma, endomeuioid type (1). SACRAMENT() (UC Davis) • Adenofibroma with low malignant potential, clear cell type, ovary. LONG BEACH - Adenofibroma (6) BAY AREA • Adenocarcinoma, ovary (3) (endomeuioid?, serous?, Brenner?, metastatic?). MlCHIGAN (Oakwood Hospital) - Proliferating endomcuioid adenofibroma. MlCH!GAN CSL Joseph Mercv Hospital) - with sex cord elements (3); Atypical proliferating endomeuioid adenofibroma (2); Adenofibroma ( 1) . NEBRASKA - Adenofibroma. MA$SACHVSETI'S (NEMCResideoiS) - Endomeuioid carcinoma vs. metastatic carcinoma. AUSTRALIA (Sydney) • Adenofibroma.

DIAGNOSIS:

ENDOMETRIOID ADENOFIBROMA OF LOW MALIGNANT POTENTIAL, OVARY T87000, M90540

REFBRP.NCES: Roth LM. Czernobilsky B. and Langley FA. Endomclriold Adenophomatous lllld Cysr.adenopboma

INLAND @versicleiSan Bernardino) • Brenner gland tumor (2). BAKERSFIELD

OIAGNOSJS:

BRENNER TUMOR, OVARY T87000, M90000

REFER£NCFe~ : Ehrlicb CB and Roth LM. The Brenner Tumor. A Clinicopathologic Study of 57 Cases. Cancer 1971; 27:332-342. lhlasa RW, Adcock LL, Prem KA and De-bncr LP. The Brenner Tumor. A Clinicopathologic Review. Obstet Gynecol 1977; 50:120-128. Bransilver BR, Fercnczy A and Ricbart RM. Brenner Tumors and· Waltbard Nests. Arch PaJho/1974; 98:76-86. Santini D. and Gelli MC, Maz:zoleni G. Ricci M. c1 al. Brenner Tumor of the Ovary. A Correlative His10logic Histocbemical: Immunobistochernical, and Ultr•·SoructUTallnvesugation. Hum PaJho/1989; 20:787-795. Martin AR. Kotylo PK. Kennedy JC, et al. Aow Cytomcuic DNA Analysis of Ovarian Brenner Tumors and Transitional Cell Carcinomas. lnt J Gyneccl Paiho/1992; II: 188-196. Silverberg SG. Brenner Tumor of the Ovary. A Clinicopathologic Study of 60 Tumors in 54 Women. Cancer 1971: 28:588-596. CASE NO. 7, ACCESSION NO. 28201 SEPTEMBER 1998

INLAND (Riverside/San Bernardino) - Serous cysladenocarcinoma, ovary (I); Well-differenlia!Cd endomctrioid carcinoma (1). BAKERSFIELD (San Joaquin Comm Hospital) - Struma ovarii (1); Struma ovarii with adenomatous changes(!). BAKERSFIELD (Central Vallev Study Grouol - Struma,ovarii. VENTURA (Unilabl - Struma ovarii (2). SANTA ROSA - Struma ovarii (3). SACRAMENTO (UC Davis) - Struma ovarii showing multinodular changes with adenoma vs adcnomatoid nodule. LONG BEACH - Struma ovarii (6). BAY AREA - Struma ovarii, left ovary (3). MICHIGAN (Oakwood Hospital) - Stnllna ovarii. MICHIGAN CSL Josenb Mercy Hospital) - Struma ovarii (6). NEBRASKA (Cieigbton) - Stnllna ovarii. n..LINOIS - Senoli-Lcydig rumor with retiform'pattern: Struma ovarii. LOUIS lANNA CShrevenortl - Struma ovariL FLORIDA Cfallahassee) - S1ruma ovarii (4). IDAHO (IDX Path

DIAGNOSIS:

STRUMA OVARD T87000, M90900

.Dfrector's Note: Most of the tumor slid.S contained colloid-filled follicles. A few slides Ia~ked the colloid, and mimicl

REFflRENCFS: Talcnnan A. A Distinctive Gonadal Related to Gonadoblastoma. Cancer 1972, 36:1219-122A. Szyfclbern WM, Young RH and Scully RE. Cystic S!r'Wna Ovarii. A 1'\'e:ed Tumor. A Report of 20 Cases. Am J Surg Pa~holl994·; 18(8):785-788. Szyfetbein Wm and Young RH. S!r'Wna Ovarii Simulating Tumor of Other Types. Am J Surg Pa1hol 1995; 19(1):21- 29. CASE NO. 8, ACCESSION NO. 28214 SEPTEMBER 1998

INLAND

DIAGNOSIS:

GONADOBLASTOMA, BILATERAL OVARIES T87000, M90731

REFERENCES: Sc·ully RE. Gonadoblastoma. A Review of 74 Case<. Cancer 1970; 25: 1340·1356. Scully RE. Gonadobla.stoma. A Gonadal Tumor Related to Dysgerminoma () Capable of~« Hormone Production. Canur 1953; 6:455463. PinkertOn IHM. McKay DG. Adam.< llC and Herteg AT. Development of the Human Ovary, A Study Using Histochemical Technics. Obsrer Gyneco1196J; 18:152-181. Gruenwald P. The Development of the Sex Cord In lbe Gonad of Man and Mammal.s Am J AIUll 1942: 70:359·389. Arroyo JG, Harris W. and Laden A. Recurrent Mixed Germ Cell Sex Cord-Stromal Tumor of the Ovary in an Adult Ins I Gynecol PaJholl998; 17:281-283. Tavassoli FA. A Combination Germ Coli Gonadal Stromal Epithelial Tumor of the Ovary. Am I Surg PaJhol1983, 7:73·84. CASE NO. 9, ACCESSiON NO. 28251 SEPTEMBER 1998

INLAND

DIAGNOSIS:

ALVEOLAR RHABDOMYOSARCOMA, UTERUS T82000, M89203

FOLLOW-UP: The patient was started 011 an aggressive regimen of chemotherapy.

REPERENCES: Parham DM. Shapiro ON, Dowing JR, et al. Solid Alveolar Rbal;domyosarcomas with the t (2: 13). Report of Two Cases with Diagnostic Implications. Am J Surg Pathcl1994; 18(5):474-478. Sbakfeh ·SM and Woodruff 10. Primary Ovarian Sarcoma. A Report of 46 Cases and Review of the literature. Obstet Gynecol Surv 1987:42:331-349. Asmar L. Gehan EA, Newton W A, et.ol. Agreement Among and Within Groups pf Pathologists in the Classification of Rhabdomyo;an:Oma and Relared Childhood Sarcomas. Report of an International Smdy of Four Pathology Classifications. CMcer 1994; 74:2579-2581l. Gray GF, .Glick AD, Kulrin PJ. et ol. AJve6lar Soft Part Sarcoma of the Uterus, Hum Pcithol 1986: 17:297-300. Foscbini MP and Eusebi V. Alveolar Soft-Part Sarcoma. A New Type of Rhabdomyosarcoma. Sem Diag Patho11994; 11(1):58-68. CASE NO. 10, ACCESSION NO, 28240 SEPTEMBER 1998

INLAND CRiversideJSan Bernardino) - Undifferentiated adenocarcinoma (1); Poorly differentiated endomctrioid carcinoma (1). B AK.ERSFJELD (San Joaquin Comm HospiiJll) - Embryonal carcinoma (1 ): Undifferentiated carcinoma (!). BAKERSFIELD (Central Valley Study Group) - Dysgenninoma vs. poorly differentiated adenocarcinoma. VENTURA - Malignant mixed mullerian tumor. KENIUCKY

DIAGNOSIS:

CARCINOSARCOMA, OVARY T87000, M89803

REEBRENCF.S: Pieretti M, Powell DE. Gallion HH. et al. Genetic Alterations of Chromosome 17 Distinguish Different Types oi Epithelial &. Hum Paiholl995; 26:393-397. Meden H, Marx D. Rocgglen T. eta!. Over Expression of the Oncogene c-erb ~ -2 (HER 2/NEU) and Re~ponse to Chemotherapy in Patients with . /nt 1 Gynect>/1998; 17:66-74. Wehrli BM. Orajewski S, G8SC.ohlstochemical Analysis of BCL-2, BAX, MCL-.1, and BCL-X Expression in Ovarian Surface Epithelial Tumcm. Iru J Gynecol Piuhol1998; 17:255-260. Czemabilslcy, Gillespie JJ and Roth LM. Adeno&areoma of the Ovary. A Light and Eleetton Microscropic Study with View of the Literature. Diag Gynecol Obstet 1982; 4:25. Wick MR and Scbwanson PE. Carcinosarcoma. Cum:nt Perspectives and an Historical Review of Nasological Concepts. SemDiag PaJho/1993; 10(2):118-127.