{"paper_id":"8451c68c-4dc3-415f-a223-d84c3eca9fdc","body_text":"Case Report\nJ Gynecol Oncol Vol. 20, No. 1:60-62, March 2009  DOI:10.3802/jgo.2009.20.1.60\n60\nA case of clear cell carcinoma arising from \nthe endometriosis of the paraovarian cyst\nJung-Yun Lee1, Eun Seon Im1, Sang-Wook Kim1, Haeryoung Kim2, Yong-Beom Kim1, Yong-Tark Jeon1\nDepartments of 1Obstetrics and Gynecology, 2Pathology, Seoul National University Bundang Hospital, Seongnam, Korea\nMalignant transformation of endometriosis is an infrequent complication. Clear cell carcinoma from endometriosis is \nvery rare in the paraovarian cyst. T o date no cases have been reported. We report a case of clear cell carcinoma arising \nfrom endometriosis of the paraovarian cyst with a brief review of literature.\nKey Words: Clear cell carcinoma, Endometriosis, Paraovarian cyst\nReceived June 28, 2008, Revised September 24, 2008,\nAccepted September 30, 2008\nAddress reprint requests to Yong-Tark Jeon\nDepartment of Obstetrics and Gynecology, Seoul National University\nBundang Hospital, 300, Gumi-dong, Bundang-gu, Seongnam 463-707, \nKorea\nTel: 82-31-787-7255, Fax: 82-31-787-4054 \nE-mail: asidof@snubh.org\nFig. 1. Computed tomography shows 16 cm sized huge cystic mass \nwith small solid portion (arrow) in the pelvic cavity.\nINTRODUCTION\nSince the first description of adenocarcinoma arising in the \nsetting of endometriosis in 1925 by Sampson,1 the malignant \ntransformation of endometriosis has been described in nu-\nmerous case reports and review of the literatures. About \n0.7-1.0% of patients with endometriosis have lesions that un-\ndergo malignant transformation.\n2,3\nIn 79% of the cases, the ovary is the primary site of malig-\nnancy, whereas extragonadal sites represent 21% of tumors.4 \nThere has been 1 documented case of clear cell carcinoma aris-\ning in vulva endometriosis.\n5 Clear cell carcinoma from endo-\nmetriosis is very rare in the paraovarian cyst and to date no \ncases have been reported. We report a case of clear cell carci-\nnoma from endometriosis of the paraovarian cyst with a brief \nreview of literatures.\nCASE REPORT\nA 62-year old, para 3, married female consulted to our in-\nstitution in May 2008 with complaints of abdominal dis-\ncomfort for 1 month. A pelvic examination revealed a normal \nsized uterus and large pelvic mass. Computed tomography \nshowed a 16 cm sized cystic mass on the right adnexa with en-\nhanced solid component (Fig. 1). Her past medical and surgical \nhistory was unremarkable. She experienced menopause at 49 \nyears old and did not take any hormone replacement therapy.\nPreoperative laboratory studies revealed the following values: \nHb 10 g/dl, WBC count 4,600/mm\n3, platelet count 324,000/ \nmm3. The results of other investigations, including urinalysis, \nliver function tests, renal function tests, electrolytes, chest ra-\ndiography, and electrocardiography revealed no abnormalities. \nTumor markers were slightly elevated as follows; CA125, 67 \nU/ml (0-48); CA19-9, 58 U/ml (0-37); CEA, ＜1.0 ng/ml (0-5). \nAt the time of surgery, the pelvic mass was a cystic lesion ad-\nhesive to the right salpinx, uterine fundus, rectum and right \nretroperitoneum. Cyst surface was clear and well marginated \nwith serous fluid inside. Uterus was bicornuate shaped and \nboth adnexae were grossly free. There was no suspicious ma-\nlignant lesions in the pelvis by inspection and palpation. Under \nthe impression of a paraovarian cyst, cyst excision and washing \n\nA case of clear cell carcinoma arising from the endometriosis of the paraovarian cyst\n61\nFig. 2. A unilocular cyst is seen with focal areas of solid papillary growth.\nThe remaining internal lining surface shows areas of hemorrhage.\nFig. 3. Scanning of the cyst with low magnification reveals a portion \nof solid growth. The cyst wall contains a smooth muscle layer and \nthe internal surface is focally lined by cuboidal to columnar epi-\nthelium (Hematoxylin-Eosin, original magnification ×10).\nFig. 4. Higher power magnification of the solid portion reveals tu-\nmor cells with pleomorphic nuclei and eosinophilic or clear cyto-\nplasm arranged in tubular or acinar structures. A hobnail pattern is \nprominent (Hematoxylin-Eosin, original magnification ×200).\ncytology was done. There was minimal intraoperative spillage. \nThe patient had an uncomplicated post-operative course.\nOn pathologic gross examination, there was a 12×7.5×5.5 \ncm cyst with white colored capsule and no infiltration of the \ncapsule. A unilocular cyst was seen with focal areas of solid \npapillary growth. The remaining internal lining surface \nshowed areas of hemorrhage (Fig. 2). Microscopic findings re-\nvealed a clear cell carcinoma arising from endometriosis with \nlocalization in the inner side of the capsule. There was no en-\ndolymphatic tumor emboli. The cyst wall contained a smooth \nmuscle layer and the internal surface was focally lined by cu-\nboidal to columnar epithelium and endometrial stromal cells. \nThis suggested an endometriosis arising from the uterine ad-\nnexa (Fig. 3). Higher power magnification of the solid portion \nrevealed tumor cells with pleomorphic nuclei and eosino-\nphilic or clear cytoplasm arranged in tubular or acinar struc-\ntures. A hobnail pattern was prominent. This coincided with \nthe typical clear cell morphology (Fig. 4). \nThe patient underwent re-operation for surgical staging with \ntotal abdominal hysterectomy, bilateral salpingo-oopho-\nrectomy, omentectomy, pelvic lymph node dissection, appen-\ndectomy, and multiple random peritoneal biopsies. On patho-\nlogic examination, there was no tumor in any specimens. \nEndometriosis was found in the right ovary. Post-operative \nhospital course was uneventful and the decision was made to \nrecommend adjuvant chemotherapy with paclitaxel and \ncarboplatin.\nDISCUSSION\nMalignant transformation of endometriosis in gonadal and \nextragonadal sites have been well documented since Sampson \nreported the first case in 1925.\n1 The criteria presented by \nSampson for diagnosis of a malignancy arising in endome-\ntriosis are: 1) demonstration of both cancerous and benign en-\ndometrial tissue in the same ovary, 2) demonstration of can-\ncer arising in the tissue and not invading it from another \nsource, and 3) presence of tissue resembling endometrial \nstroma surrounding characteristic epithelial glands.\n1\nThe estimated prevalence of endometriosis are 15% of pre-\nmenopausal and 2-5% of postmenopausal women.6 However, \nabout 0.7-1.0% of patients with endometriosis have lesions \nthat undergo malignant transformation.\n2,3 Heap et al. 4 re-\nviewed 205 reported cases of malignancy arising in endome-\ntriosis and reported that the ovary was the most frequently in-\nvolved, accounting for 165 cases (78.7%), and that extra-\ngonadal tumors were present in 44 (21.3%). The rectovaginal \nseptum, colon, vagina, and pelvic peritoneum represented the \nmajority of extragonadal sites. Irvin et al.\n7 also reviewed 222 \nreported cases of malignancy arising in endometriosis, and re-\nported that the ovary was most frequently involved, account-\ning for 169 cases (76%), and extragonadal tumors were 53 \n\nJ Gynecol Oncol Vol. 20, No. 1:60-62, 2009 Jung-Yun Lee, et al.\n62\n(24%). This concords with the frequency of endometriosis ac-\ncording to location.\nPelvic pain or pelvic mass in a postmenopausal woman with \na previous history of endometriosis should raise suspicions of \nreactivation or malignant transformation of endometriosis. \nVaginal bleeding may signify the presence of a vaginal or rec-\ntovaginal septum lesion, and the bleeding may be caused by \nestrogenic stimulation. Malignant transformation of color-\nectal endometriosis may produce gastrointestinal dysfunction \nand/or bleeding.\n8-10 Urinary symptoms may herald urinary \ntract involvement with this disease.11,12\nWhen malignant transformation occurs in endometriosis, it \ntends to be discovered at an early stage and is often of low \ngrade. Primary surgical treatment with complete resection of \npelvic tumors should be performed when feasible. Appropriate \nstaging biopsies of lymph nodes and tissues in the upper ab-\ndomen should be performed when macroscopic disease is \nconfined to pelvis.\n4\nAure et al.13 noted that the prognosis and 5-year survival were \nthe same, stage for stage, as those for patients with ovarian \ncarcinoma. Malignant transformation within endometriomas \nor within extragonadal endometriosis confined to the genital \ntract carries a much better prognosis, with a 67% 5-year sur-\nvival for those with disease confined to the ovary and 100% \n5-year survival for those with extragonadal disease confined to \nthe site of the origin. Disseminated intraperitoneal disease \nhad a poor prognosis, with a 12% 5-year survival.\n4\nAmong malignancies arising from endometriosis of the ova-\nries or extragonadal lesions, endometrioid adenocarcinoma \nwas the most common histologic type (69.1%). Clear cell his-\ntology was seen in only 4.5% of extragonadal malignancies.\n4 \nTo our knowledge, this is the first case of clear cell carcinoma \narising from endometriosis of the paraovarian cyst. \nREFERENCES\n1. Sampson JA. Endometrial carcinoma of ovary, arising endo-\nmetrial tissue in that organ. Arch Surg 1925; 10: 1-72.\n2. Corner GW , Hu CY , Hertig AT . Ovarian carcinoma arising in \nendometriosis. Am J Obstet Gynecol 1950; 59: 760-74.\n3. Lauslahti K. Malignant external endometriosis: a case of ad-\nenocarcinoma of umbilical endometriosis. Acta Pathol Microbiol \nScand Suppl 1972; 233: 98-102.\n4. Heaps JM, Nieberg RK, Berek JS. Malignant neoplasm arising \nin endometriosis. Obstet Gynecol 1990; 75: 1023-8.\n5. Cho HJ, Lee KJ, Cha SH, Seong SJ, Park CT , Lee KH, et al. Clear \ncell carcinoma of the vulva arising in endometriosis. Korean J \nObstet Gynecol 2003; 46: 847-50.\n6. Punnonen R, Klemi PJ, Nikkanen V . Postmenopausal endometriosis. \nEur J Obstet Gynecol Reprod Biol 1980; 11: 195-206.\n7 . I r v i n  W ,  P e l k e y  T ,  R i c e  L ,  A n d e r s e n  W .  E n d o m e t r i a l  s t r o m a l  \nsarcoma of the vulva arising in extraovarian endometriosis: a \ncase report and literature review. Gynecol Oncol 1998; 71: \n313-6.\n8. Reintoft I, Lange AP , Skipper A. Coincidence of granulosa-cell \ntumour of ovary and development of carcinoma in rectal \nendometriosis. Acta Obstet Gynecol Scand 1974; 53: 185-9.\n9. Amano S, Yamada N. Endometrioid carcinoma arising from en-\ndometriosis of the sigmoid colon: a case report. Hum Pathol \n1981; 12: 845-8.\n10. Amano S, Yamada N. Endometrioid carcinoma arising from en-\ndometriosis of the sigmoid colon: a case report. Hum Pathol \n1981; 12: 845-8.\n11. Shamsuddin AK, Villa Santa U, T ang CK, Mohamed NC. \nAdenocarcinoma arising from extragonadal endometriosis 14 \nyears after total hysterectomy and bilateral salpingo-oopho-\nrectomy for endometriosis: report of a case with ultrastructural \nstudies. Am J Obstet Gynecol 1979; 133: 585-6.\n12. Kapadia SB, Russak RR, O'Donnell WF , Harris RN, Lecky JW . \nPostmenopausal ureteral endometriosis with atypical ad-\nenomatous hyperplasia following hysterectomy, bilateral \noophorectomy, and long-term estrogen therapy. Obstet Gynecol \n1984; 64(3 Suppl): 60S-3S.\n13. Aure JC, Hoeg K, Kolastad P . Carcinoma of the ovary and \nendometriosis. Acta Obstet Gynecol Scand 1971; 50: 63-7.","source_license":"CC0","license_restricted":false}