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A Rare Case of Multiple Malignancies arising in Endometriosis: Diffuse Extra-Uterine High-grade Endometrial Stromal Sarcoma and Ovarian Carcinoma

Jolene Wong, Grace Tan, Claramae Chia, Sathiyamoorthy Selvarajan, Melissa Teo


Introduction: Malignant transformation of endometriotic foci is a rare event occurring in 0.7-1% of patients with endometriosis. Of neoplasms that develop, endometrial stromal sarcoma (ESS) has been described and represent an extremely rare histological subtype [1].  Since its first description in the 1920s, only a handful of case reports have been published. The gastrointestinal tract is the most  common site for endometriosis associated ESS, although cases of ESS involving the mesentery, omentum, ovary, vagina, cervix, rectovaginal septum, parametrium, fallopian tube, pelvis, retroperitoneum, liver, and sciatic nerve have been reported [2-15].

Case Presentation: We present a case of diffuse metastatic high grade ESS as well as clear cell carcinoma of the ovary associated with endometriosis in a 45-year-old woman treated with complete cytoreductive surgery.

Conclusion: The development of multiple malignancies arising from disseminated endometriotic foci in the abdominal cavity represents an extremely rare event. A high index of clinical suspicion is required to reach a diagnosis. Furthermore, no gold standard management option exists and treatment should be individualized.


Endometrial stromal sarcoma; Endometriosis; Ovarian clear cell carcinoma

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Heaps JM, Nieberg RK, Berek JS. Malignant neoplasms arising in endometriosis. Obstet Gynecol. 1990, 75:1023-1028

Khan AW, Craig M, Jarmulowicz M, Davidson BR. Liver tumours due to endometriosis and endometrial stromal sarcoma. HPB. 2002, 4:43-46

Cho HY, Kim MK, Cho SJ, Bae JW, Kim I. Endometrial stromal sarcoma of the sigmoid colon arising in endometriosis: a case report with a review of literature. J Korean Med Sci. 2002, 17:412-414

Baiocchi G, Kavanagh JJ, Wharton JT. Endometrioid stromal sarcomas arising from ovarian and extraovarian endometriosis: report of two cases and review of the literature. Gynecol Oncol. 1990, 36:147-151

Corpa MV, Serafini EP, Bacchi CE. Low-grade endometrial stromal sarcoma presenting as vaginal nodule. Ann Diagn Pathol. 2004, 8: 295-298

Bosincu L, Massarelli G, Cossu Rocca P, Isaac MA, Nogales FF. Rectal endometrial stromal sarcoma arising in endometriosis. Dis Colon Rectum. 2001, 44:890-892

Fukunaga M, Ishihara A, Ushigome S. Extrauterine low-grade endometrial stromal sarcoma: report of three cases. Pathol Int. 1998, 48:297-302

Ferraro LR, Hetz H, Carter H. Pelvic endometriosis complicated by polypoid endometrioma of the colon and endometriotic sarcoma; report of a case and review of the literature. Obstet Gynecol. 1956, 7: 32-39

Kovac D, Gasparović I, Jasic M, Fuckar D, Dobi-Babić R, Haller H. Endometrial stromal sarcoma arising in extrauterine endometriosis: a case report. Eur J Gynaecol Oncol. 2005, 26:113-116

Rojas H, Wang J, Chase D, et al. Pathologic quiz case: a 46-year-old woman with 1-day history of abdominal pain and intestinal obstruction. Arch Pathol Lab Med. 2005, 129:e44-6

Lacroix-Triki M, Beyris L, Martel P, Marques B. Low-grade endometrial stromal sarcoma arising from sciatic nerve endometriosis. Obstet Gynecol. 2004, 104:1147-1149

Mourra N, Tiret E, Parc Y, de Saint-Maur P, Parc R, Flejou JF. Endometrial stromal sarcoma of the rectosigmoid colon arising in extragonadal endometriosis and revealed by portal vein thrombosis. Arch Pathol Lab Med. 2001, 125:1088-1090

Son HJ, Kim JH, Kang DW, Lee HK, Park MJ, Lee SY. Primary extrauterine endometrial stromal sarcoma in the sigmoid colon. Ann Coloproctol. 2015, 31(2):68-73

Yantiss RK, Clement PB, Young RH. Neoplastic and pre-neoplastic changes in gastrointestinal endometriosis: a study of 17 cases. Am J Surg Patho. 2000, 24:513-524

Morrison C, Ramirez NC, Chan JK, Wakely P. Endometrial stromal sarcoma of the retroperitoneum. Ann Diagn Pathol. 2002, 6:312-318

Palladino VS, Trousdell M. Extra-uterine mullerian tumors: a review of the literature and the report of a case. Cancer. 1969, 23:1413-1422

Sampson JA. Metastatic or embolic endometriosis, due to the menstrual dissemination of endometrial tissue into the venous circulation. Am J Pathol. 1927, 3:93-110.43

Lauchlan SC. The secondary Miillenan system. GWet. Gynewl. Survey 1972, 27: 133-146

Alczar JL, Guerriero S, Ajossa S, Parodo G, Piras B, Peiretti M, Jurado M, Idoate M. Extragenital endometrial stromal sarcoma arising in endometriosis. Gynecol Obstet Invest. 2012, 73(4):265-271

Young RH, Prat J, Scully RE. Endometrioid stromal sarcomas of the ovary. A clinicopathologic analysis of 23 cases. Cancer. 1984, 53(5):1143-1155

Mostoufizadeh M, Scully RE. Malignant tumors arising in endometriosis. Clin Obstet Gynecol. 1980, 23(3):951-63. PubMed ID: 7418292

Biliatis I, Akrivos N, Sotiropoulou M, Rodolakis A, Simou M, Antsaklis A. Endometrial stromal sarcoma arising from endometriosis of the terminal ileum: the role of immunohistochemistry in the differential diagnosis. J Obstet Gynaecol Res. 2012, 38(5):899-902

Swiersz LM. Role of endometriosis in cancer and tumor development. Ann N Y Acad Sci. 2002, 955:281-292

Kim HS, Kim TH, Chung HH, Song YS. Risk and prognosis ofovarian cancer in women with endometriosis: a meta-analysis. Br J Cancer. 2014, 110:1878-1890

Pearce CL, Templeman C, Rossing MA, Lee A, Near AM, Webb PM, Nagle CM, Doherty JA, Cushing-Haugen KL, Wicklund KG, Chang-Claude J, Hein R, Lurie G, Wilkens LR, Carney ME, Goodman MT, Moysich K, Kjaer SK, Hogdall E, Jensen A, Goode EL, Fridley BL, Larson MC, Schildkraut JM, Palmieri RT, Cramer DW, Terry KL, Vitonis AF, Titus LJ, Ziogas A, Brewster W, Anton-Culver H, Gentry-Maharaj A, Ramus SJ, Anderson AR, Brueggmann D, Fasching PA, Gayther SA, Huntsman DG, Menon U, Ness RB, Pike MC, Risch H, Wu AH, Berchuck A, Ovarian Cancer Association Consortium. Association between endometriosis and risk of histological subtypes of ovarian cancer: a pooled analysis of case-control studies. Lancet Oncol. 2012, 13:385-394

Weitmann HD, Knocke TH, Kucera H. Radiation therapy in the treatment of endometrial stromal sarcoma. Int J Radiat Oncol Biol Phys. 2001, 49(3):739-748

Mansi JL, Ramachandra S, Wiltshaw E, Fisher C. Endometrial stromal sarcomas. Gynecol Oncol. 1990, 36(1):113-118

Norris HJ, Taylor HB. Post-irradiation sarcomas of the uterus. Obstet Gynecol. 1965, 26(5):689-694

Garg G, Awonuga AO, Toy EP. Primary extrauterine endometrial stromal sarcoma: response to hormone therapy. Rare Tumors. 2009, 1(2):e39

Sugarbaker PH. New strands of care for appendiceal neoplasms and pseudomyxoma peritonei syndorome? Lancet Oncol. 2006, 7: 69-76

Bakrin N, Gilly FN, Baratti D, Bereder JM, Quenet F, Lorimier G, Mohamed F, Elias D, Glehen O, Association Franaise de Chirurgie. Primary peritoneal serous carcinoma treated by cytore- ductive surgery combined with hyperthermic intraperitoneal chemotherapy. A multi- institutional study of 36 patients. Eur J Surg Oncol. 2013, 39:742-747

Bakrin N, Cotte E, Golfier F, Gilly FN, Freyer G, Helm W, Glehen O, Bereder JM. Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC) for persistent and recurrent advanced ovarian carcinoma: a multi- center, prospective study of 246 patients. Ann Surg Oncol. 2012, 19:4052-4058

Jimenez WA, Sardi A, Nieroda C, Gushchin V. Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy in the management of recurrent high-grade uterine sarcoma with peritoneal dissemination. Am J Obstet Gynecol. 2014, 210(3):259.e1-8


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