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Endometrial stromal sarcomas (ESS) are rare malignant tumors of the uterus accounts for 0.2 of all genital tract malignancies. They occur more often in premenopausal women. Most of the information available in literature is based on small series or case reports hence there are limited              information regarding the clinical management and final           outcome of the disease. Non-puerperal inversion of the uterus is an uncommon presentation of ESS. We report this case of ESS with inversion of uterus in a 23 year old unmarried girl due to its rarity of clinical presentation. She presented with abnormal uterine bleeding associated with abdominal pain and mass descending PV. On examination she was obese, anemic and no masses palpable per abdomen. Local            examination showed a large mass lying outside the introitus with foul smelling blood stained vaginal discharge. Biopsy showed a high grade endometrial stromal sarcoma. With the diagnosis of high grade ESS - stage IIB with inversion of uterus, she underwent surgical management - abdomino vaginal approach. Histopathology confirmed the diagnosis of high grade ESS. She received postoperative adjuvant         radiotherapy in view of extra-uterine disease. Endometrial stromal sarcomas are aggressive tumors with poor prognosis. The 5 year disease free survival is 25. Total hysterectomy, bilateral salpingooophorectomy with pelvic lymphadenectomy is the optimal treatment in cases of ESS. Ovarian                                     conservation may be possible in young women and with early stage disease and the role of lymphadenectomy is                   controversial. Adjuvant therapy is useful to prevent recurrence of disease but their role in survival is questionable.


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