The diagnosis rate of deep pelvic endometriosis is increasing. Endometrial stromal sarcoma (ESS) is a rare neoplasm. Extragenital ESS is an extremely uncommon event. Very few cases of extragenital ESS have been reported to date. The diagnosis of this entity is very difficult in some instances. Knowledge about its management is also limited. In this paper, we review the current literature on the clinical management, histology, immunohistochemistry, treatment and outcome of ESS arising in pelvic endometriosis.

Heaps JM, Nieberg RK, Berek JS: Malignant neoplasms arising in endometriosis. Obstet Gynecol 1990;75:1023–1028.
Stern RC, Dash R, Bentley RC, Snyder MJ, Haney AF, Robboy SJ: Malignancy in endometriosis: frequency and comparison of ovarian and extraovarian types. Int J Gynecol Pathol 2001;20:133–139.
Heaps JM, Nieberg RK, Berek JS: Malignant neoplasms arising in endometriosis. Obstet Gynecol 1990;75:1023–1028.
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
Persaud V, Anderson MF: Endometrial stromal sarcoma of the broad ligament arising in an area of endometriosis in a paramesonephric cyst. Case report. Br J Obstet Gynecol 1977;84:149–152.
Berkowitz RS, Ehrmann RL, Knapp RC: Endometrial stromal sarcoma arising from vaginal endometriosis. Obstet Gynecol 1978;51:34–37.
Young RH, Prat J, Scully RE: Endometrial sacromas of the ovary. A clinicopathologic analysis of 23 cases. Cancer 1984;53:1143–1155.
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.
Vierhout ME, Chadha-Ajwani S, Wijnen JA, Splinter TAW, Zondervan PE, Ras J, Drogendijk AC: Extra-uterine endometrial stromal sarcoma with DNA flow cytometric analysis. Eur J Obstet Gynecol Reprod Biol 1992;43:157–161.
Chang KL, Crabtree GS, Lim-Tan SK, Kempson RL, Hendrickson MR: Primary extrauterine endometrial stromal neoplasms: a clinicopathologic study of 20 cases and a review of the literature. Int J Gynecol Pathol 1993;12:282–296.
Fishman A, Demirel D, Laucirica R, Ramzy I, Klima T, Lyzak J, Kaplan AL: Malignant tumors arising in endometriosis: clinical-pathological study and flow cytometry analysis. Eur J Obstet Gynecol Reprod Biol 1996;70:69–74.
Kondi-Paphitis A, Smyrniotis B, Liapis A, Kontoyanni A, Deligeorgi H: Stromal sarcoma arising on endometriosis. A clinicopathological and immunohistochemical study of 4 cases. Eur J Gynaecol Oncol 1998;19:588–590.
Irvin W, Pelkey T, Rice L, Andersen W: Endometrial stromal sarcoma of the vulva arising in extraovarian endometriosis. A case report and literature review. Gynecol Oncol 1998;71:313–316.
Fukunaga M, Ishihara A, Ushigome S: Extrauterine low-grade endometrial stromal sarcoma: report of three cases. Pathol Int 1998;48:297–302.
Yantiss RK, Clement PB, Young RH: Neoplastic and preneoplastic changes in gastrointestinal endometriosis. A study of 17 cases. Am J Surg Pathol 2000;24:513–524.
Levine PH, Abou-Nassar S, Mittal K: Extrauterine low-grade endometrial stromal sarcoma with florid endometrioid glandular differentiation. Int J Gynecol Pathol 2001;20:395–398.
Mourra N, Tiret E, Parc Y, 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.
Bosincu L, Massarelli G, Cossu Rocca P, Isaac MA, Nogales FF: Rectal endometrial stromal sarcoma arising in endometriosis: report of a case. Dis Colon Rectum 2001;44:890–892.
Khan AW, Craig M, Jarmulowicz M, Davidson BR: Liver tumours due to endometriosis and endometrial stromal sarcoma. HPB (Oxford) 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 literatures. J Korean Med Sci 2002;17:412–414.
Geas FL, Tewari DS, Rutgers JK, Tewari KS, Berman ML: Surgical cytoreduction and hormone therapy of an advanced endometrial stromal sarcoma of the ovary. Obstet Gynecol 2004;103:1051–1054.
Corpa MV, Serafini EP, Bacchi CE: Low-grade endometrial stromal sarcoma presenting as vaginal nodule. Ann Diagn Pathol 2004;8:295–298.
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.
Mitchard JR, Lott M, Afifi RA, Hirschowitz L: Low-grade endometrial stromal sarcoma with glandular differentiation arising in ovarian endometriosis. J Obstet Gynaecol 2004;24:596–597.
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.
Kusaka M, Mikuni M, Nishiya M: A case of high-grade endometrial stromal sarcoma arising from endometriosis in the cul-de-sac. Int J Gynecol Cancer 2006;16:895–899.
Hasiakos D, Papakonstantinou K, Kondi-Paphiti A, Fotiou S: Low-grade endometrial stromal sarcoma of the endocervix. Report of a case and review of the literature. Eur J Gynaecol Oncol 2007;28:483–486.
Chen CW, Ou JJ, Wu CC, Hsiao CW, Cheng MF, Jao SW: High-grade endometrial stromal sarcoma arising from colon endometriosis. Int J Colorectal Dis 2007;22:1551–1553.
Budäus LH, Menzel T, Hartmann V, Caselitz J, Doerner A: Acute abdomen caused by endometrial stromal sarcoma arising in extragonadal foci of endometriosis of the terminal ileum. Int J Colorectal Dis 2008;23:447–448.
Kim L, Choi SJ, Park IS, Han JY, Kim JM, Chu YC, Kim KR: Endometrial stromal sarcoma of the small bowel. Ann Diagn Pathol 2008;12:128–133.
Zemlyak A, Hwang S, Chalas E, Pameijer CR: Primary extrauterine endometrial stromal cell sarcoma: a case and review. J Gastrointest Cancer 2008;39:104–106.
Jung SI, Shin SS, Choi C, Hwang EC, Kim SO, Kang TW: Endometrial stromal sarcoma presenting as prevesical mass mimicking urachal tumor. J Korean Med Sci 2009;24:529–531.
Kim JY, Hong SY, Sung HJ, Oh HK, Koh SB: A case of multiple metastatic low-grade endometrial stromal sarcoma arising from an ovarian endometriotic lesion. J Gynecol Oncol 2009;20:12212–12215.
Doghri R, Mrad K, Driss M, Sassi S, Abbes I, Dhouib R, Hechiche M, Romdhane KB: Endometrial stromal sarcoma presenting as a cystic abdominal mass. Pathologica 2009;101:93–96.
Sinha R, Sundaram M: Endometrial stromal sarcoma from endometriosis. J Minim Invasive Gynecol 2010;17:541–542.
Roşca E, Venter A, Muţiu G, Drăgan A, Coroi M, Roşca DM: Endometrial stromal sarcoma developed on outer endometriosis foci. Rom J Morphol Embryol 2011;52:489–492.
McCluggage WG, Bailie C, Weir P, Bharucha H: Endometrial stromal sarcoma arising in pelvic endometriosis in a patient receiving unopposed oestrogen therapy. Br J Obstet Gynaecol 1996;103:1252–1254.
Zaloudek C, Norres HJ: Mesenchymal tumors of the uterus; in Kurmann RJ (ed): Blaunstein’s Pathology of the Female Genital Tract, ed 3. New York, Springer, 1987.
Moinfar F, Azodi M, Tavassoli FA: Uterine sarcomas. Pathology 2007;39:55–57.
Abeler VM, Nenodovic M: Diagnostic immunohistochemistry in uterine sarcomas: a study of 397 cases. Int J Gynecol Pathol 2011;30:236–243.
Jung CK, Jung JH, Lee A, Lee YS, Choi YJ, Yoon SK, Lee KY: Diagnostic use of nuclear beta-catenin expression for the assessment of endometrial stromal tumors. Mod Pathol 2008;21:756–763.
Klein WM, Kurman RJ: Lack of expression of c-kit protein (CD117) in mesenchymal tumors of the uterus and ovary. Int J Gynecol Pathol 2003;22:181–184.
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