腎細胞癌と腎血管筋脂肪腫を合併した結節性硬化症の1例

Abstract

The coincidence of renal cell carcinoma and renal angiomyolipoma in tuberous sclerosis is extremely rare, although the coexistence of tuberous sclerosis and renal angiomyolipoma is well recognized. A case of bilateral renal angiomyolipomas and left renal cell carcinoma in a patient with tuberous sclerosis is reported. A 40-year-old male was referred to our hospital for further evaluation and treatment of left flank masses. Tuberous sclerosis was diagnosed on the basis of adenoma sebaceum, seizures, mental retardation and periventricular calcification. Contrast enhanced CT scan demonstrated irregularly enhanced masses in the upper pole and the middle portion of the left kidney, and multiple small low density nodules in the bilateral kidneys. Selective left renal angiography showed hypervascular areas in the upper pole and the middle portion of the left kidney. From the findings obtained, a clinical diagnosis of left renal cell carcinoma associated with bilateral renal angiomyolipomas was made. Left radical nephrectomy was performed. The histopathological examination revealed renal cell carcinoma and multiple renal angiomyolipoma nodules. The diagnostic considerations, particularly differential diagnosis between renal angiomyolipoma and renal cell carcinoma by imaging features, are discussed.