Giant Angiomyolipoma with Epithelial Cyst (AMLEC): Case Report and Review of Literature

Angiomyolipoma with epithelial cyst (AMLEC) is a rare variant of angiomyolipoma (AML). Hereby, we report a unique case which is the largest size of AMLEC ever reported, the patient being the youngest one ever recorded, and the first case of AMLEC associated with tuberous sclerosis complex reported from India. The patient was a 19-year-old woman who presented with hematuria. Contrast-enhanced computed tomography (CT) and positron emission tomography-CT detected a large complex cystic lesion, which was reported to be AML with a less likely possibility of cystic renal cell carcinoma. The patient underwent radical nephrectomy. Histopathology and immuno-histochemistry identified the tumor to be AMLEC. On further evaluation, the patient was found to satisfy clinical criteria of tuberous sclerosis complex. Rare forms of renal tumor like AMLEC need to be kept in mind whenever we encounter complex cystic lesions in the kidney.