Primary Low-Grade Extrauterine Endometrial Stromal Sarcoma with Sex Cord Elements Involving the Omentum

Chikkanaganna P. Manjula, V. Suguna Beluru, Bharath Raju, Naina Mary Simon
In: Archives of Medicine and Health Sciences · 2022 · vol. 10(1) , pp. 87–90 · doi:10.4103/amhs.amhs_6_22 · W4285202444
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Abstract

Endometrial stromal sarcoma (ESS) is a rare mesenchymal neoplasm, accounting for 0.2% of all uterine malignancies. They also occur in extrauterine locations such as ovaries, rectal wall, pelvic peritoneum, and vagina, where it is associated with endometriosis. Here, we present a case of extrauterine ESS of the omentum, which resembled a urachal cyst on imaging studies. Intraoperatively, the bladder was normal, and multiple solid cystic nodules were noted in the omentum. Histopathological examination revealed features of spindle cell neoplasm. Immunohistochemistry helped in arriving at the final diagnosis of low-grade endometrial stromal sarcoma with sex cord-like elements. However, extensive sampling did not reveal endometriotic foci. Further investigations to search for a primary lesion in the female genital tract were futile, and patient remained asymptomatic 2 years after surgery. Like in uterine ESS, surgery remains the mainstay of treatment.

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endometriosis

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