Endometrial Stromal Sarcoma (ESS) are very rarely encountered malignancies of uterus that account for only 1% of all uterine malignancies. ESS is commonly seen in perimenopausal women, typically presents with abnormal uterine bleeding and most commonly, pre-operative diagnosis will be leiomyomas. We report an interesting case of 42 year old female presenting with irregular cycles and excessive bleeding per vaginum. With provisional clinical diagnosis of fibroid uterus, total abdominal hysterectomy was done. Histopathological examination and immunohistochemistry staining with CD 10 confirmed the diagnosis of Low Grade ESS. The case is presented in view of its rarity and showing very much distinguishing gross and microscopy appearance. It highlights the unsuspected uterine malignant tumor, which was mimicking leiomyoma both clinically and radiologically. The histopathological examination again proved as gold standard to confirm the diagnosis of this rare entity.
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