Rare Case of Endometrial Stromal Sarcoma with Omental Metastasis in a 19-Year-Old Girl

Extrauterine endometrial stromal sarcoma (ESS) is a rare entity and typified by delayed recurrence of primary ESS. Here, we present an unusual case of uterine ESS in a woman with a history of hysterectomy. A 19-year-old girl underwent hysterectomy and bilateral salpingo-oophorectomy for uterine ESS 12 months ago, and now after remaining disease-free for 9 months ago she was presented with ascites along with pelvic and peritoneal mass. Intraoperatively, large omental mass was found and optimal cytoreduction with total omentomy (supracolic and infracolic), total peritonectomy and hyperthermic intraperitoneal chemotherapy (HIPEC) was offered to the patient. Final histopathology report showed involvement of only omentum by ESS cells. IHC and receptor study was done, and it was positive for CD-10 and desmin and negative for CK-7. This case highlights the rarity of extrauterine ESS in the omentum with a known history of primary uterine ESS which was treated successfully with the above-mentioned procedure, though active and longterm surveillance is recommended to monitor for late recurrences.