Uterine angiomyolipoma: A case report of an unusual entity

Abstract

Angiomyolipoma (AML) is a rare benign mesenchymal neoplasm composed of a variable mixture of smooth muscle cells, adipose tissue and anomalous blood vessels. It belongs to the family of perivascular epithelioid cell tumors (PEComas). It is quite common in kidney but is rare at the extrarenal sites. In the uterus, only a few cases have been reported. We describe a case of uterine AML without evidence of tuberous sclerosis (TS). It was clinicoradiologically mistaken for fibroid. The clinical presentation of uterine AML is similar to more common uterine leiomyomas. AML can be suspected on the imaging but histopathology establishes a confirmed diagnosis. The case is presented for its rarity and likelihood of mistaking it for some other mesenchymal tumors. Overall, just 36 cases of Uterine AML have been reported in the English literature prior to this and this is only the second Indian case.