Epithelioid PEComa coexisting with angiomyolipoma in the kidney

Renal epithelioid perivascular epithelioid cell tumors (PEComas) are rare neoplasms with potential for aggressive clinical behaviour that present a significant diagnostic challenge due to imaging features that overlap with renal cell carcinoma (RCC). We present the case of a 51-year-old female with the coexistence of a typical fat-containing renal angiomyolipoma (AML) and an adjacent large, fat-poor renal mass on computed tomography (CT), confirmed on histopathology as an epithelioid PEComa, with high-risk features including large size and necrosis. Despite complete resection, a local recurrence of the PEComa was detected in the surgical bed 14 months postoperatively. This case serves as a clinical example of the rare but recognized association between classic AML and epithelioid PEComa. Furthermore, the recurrence also reinforces the necessity of diligent long-term surveillance following the resection of such tumors, especially in cases with high risk features.