International Journal of Clinical and Diagnostic Pathology

Endometrial stromal Sarcoma (ESS) is a rare malignant neoplasm of uterus occurring in perimenopausal age group. The annual incidence of ESS is 1–2 per million women. ESSs are morphologically heterogeneous. The distinction between uterine smooth muscle tumors such as myxoid leiomyosarcoma and low-grade ESS can be difficult when stromal sarcomas have abundant myxoid stroma. A definitive diagnosis of stromal sarcoma can be made if myometrial invasion is present, but hysterectomy is usually required for definitive diagnosis. We report four cases of ESS encountered in a period of one year all of which was confirmed by immunohistochemistry.