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A 48-year-old P2L2 female presented to the OPD with complaint of heavy menstrual bleeding since last 1 year and irregular menstrual bleeding since last 3 months. On per abdomen examination a mass of 20-22 weeks size was palpated, nontender, hard in consistency, smooth regular margins and was mobile. On per speculum examination cervix was hypertrophied and vagina was healthy. On per vaginum examination uterus was found deviated to left side and mass was felt in right adnexa which was mobile with cervical motion. Patient was followed with ultrasonography and MRI pelvis. Her ultrasound showed a heterogenous hypoechoic lesion of size 15.6 cm x 12.3 cm the uterus, right adnexa Her MRI pelvis was suggestive of degenerated uterine fibroid or cystic adenomatoid tumor. A diagnosis of AUB-L was made and patient was prepared for laparotomy. She underwent Total Abdominal Hysterectomy with bilateral salpingooophorectomy. Uterus was found enlarged (UCL 14cm) [1-2]. On cut section it showed a posterior fundal fleshy mass protruding into endometrial cavity with multilocular cystic appearance with thick septations filled with serous fluid (Figure 1).
ESS is a rare uterine neoplasm comprising only 0.2% of all uterine malignancies and about 20% of uterine sarcomas occurring primarily in perimenopausal women. Most common presentation being abnormal uterine bleeding, abdominal pain and pressure symptoms. Pelvic examination usually reveals an enlarged uterus with regular or irregular margins sometime associated with rubbery parametrial induration. Low grade ESS is diagnosed histologically based on cellular atypia, mitotic activity and vascular invasion [2-4]. About 40% cases may have an extra uterine spread at presentation but an extra pelvis spread is rare. Mainstay of treatment remains total abdominal hysterectomy with bilateral salphingoophrectomy. Pelvic irradiation or hormonal therapy with progestins or aromatase inhibitors is recommended for inadequate excised or locally recurrent pelvic disease or metastatic disease. Sections from the fundal mass is showing small, oval darkly stained cells resembling endometrial stroma in sheets and small fascicles as well as scattered in small clusters surrounding blood vessels with areas of hyalinization. The cells are seen streaming into myometrium to variable thickness. However, showing invasion into vascular channels. There is no mitosis. The tumor is extending till serosal surface however not beyond that (Figure 2).
Low grade ESS is an extremely rare neoplasm with a very good prognosis. Post-operative diagnosis is difficult and is mostly made post operatively with histopathological findings. These tumors are indolent in nature and local recurrences are common. Patient should be followed up with oncology. Recurrences and metastatic diseases yield response to treatment with hormonal therapy or pelvic irradiation.
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