Lymph nodal involvement by renal angiomyolipoma

Angiomyolipoma of the kidney is a clonal neoplasm, apparently part of a family of neoplasms derived from perivascular epithelial cells. A 40‐year‐old woman presented with right flank pain and an otherwise non‐significant medical history. An abdominal computed tomography scan revealed an 18 cm solid mass in the mid‐portion of the right kidney and multiple perihilar lymph nodes. Presumptive diagnosis was renal cell carcinoma. Right radical nephrectomy and a perihilar lymph node dissection was performed through a Chevron incision for the anticipated diagnosis of renal adenocarcinoma. The renal tumor was diagnosed as angiomyolipoma and a component was identified pathologically in a dissected lymph node. There was no evidence of tumor recurrence in the follow‐up period of eight years. The consensus from other studies suggests that this phenomenon is a manifestation of the multicentric nature of angiomyolipoma, rather than due to metastasis. Genetic studies may resolve this question in the future.