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

Jolene Si Min Wong, Grace Fangmin Tan, Claramae Shulyn Chia, Sathiyamoorthy Selvarajan, Melissa C.C. Teo
In: American journal of cancer case reports · 2015 · vol. 3(3) , pp. 135–142 · W1628814304
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This paper reports an extremely rare case of a 45-year-old woman with multiple malignancies: diffuse metastatic high-grade endometrial stromal sarcoma and ovarian clear cell carcinoma, both associated with endometriosis.

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Abstract

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.

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Condition tags

endometriosis

Citation neighborhood (2-hop)

Papers in the corpus that this work cites (lower rings, blue) and that cite this one (upper rings, green). Dot size scales with the paper's in-corpus citation count — bigger dot = more influential within the endo/adeno field. Click a dot to open that paper. Outer rings show 2-hop neighbours — papers reached through the immediate citers/citees. [ collapse to 1-hop ]

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