Renal sclerosing AML/PEComa in a male - A case report and literature review

Sclerosing angiomyolipomas (AMLs) are a subtype of perivascular epithelioid cell-omas (PEComas) with significant stromal hyalinisation and AML-like features. Immunochemically, they show myomelanocytic differentiation. Sclerosing AMLs/PEComas are predominantly retroperitoneal, follow a benign course, and are overwhelmingly reported in females. Here we report a renal sclerosing AML/PEComa in a male. This case highlights the importance of considering sclerosing AMLs/PEComas as a likely indolent differential for small renal masses in men, as accepted knowledge of small renal masses with these characteristics suggests 70–80 % will be malignant. • Sclerosing AMLs demonstrate AML-like features with myomelanocytic differentiation. • Sclerosing AMLs/PEComas follow a benign course and are predominantly reported in females. • Sclerosing AMLs/PEComas are an important indolent differential for small renal masses in men as well as women.