Sclerosing perivascular epithelioid cell tumor of the uterus: A rare entity posing diagnostic challenge

Sclerosing perivascular epithelioid cell tumor of the uterus: A rare entity posing diagnostic challenge

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Perivascular epithelioid cell tumor Water Inlet Nozzle (PEComa) is a rare mesenchymal uterine tumor and the histological variant, sclerosing PEComa is exceedingly rare.Sclerosing PEComas preferentially occur in the retroperitoneum and occurrence in the uterine corpus is seldom seen.These tumors pose a diagnostic challenge and need distinction from morphological mimickers such as epithelioid smooth muscle tumors, endometrial stromal sarcoma, and metastatic carcinoma.Accurate diagnosis can be 3-Layer Wooden Ornament established coupling histomorphology with immunostaining.

The distinction from other entities is of prime importance considering the therapeutic and prognostic implications.Herein, we describe a case of uterine sclerosing variant of PEComa with diagnostic difficulties and key to diagnose this entity.