結節性硬化症の不全型と考えられる両側腎血管筋脂肪腫の1例

Abstract

A 39-year-old woman was admitted with the chief complaints of abdominal mass and pain in the right flank. Preoperative diagnosis was made as bilateral renal angiomyolipoma from clinical symptoms and angiograms. The patient underwent bilateral partial nephrectomy and the above diagnosis was confirmed. Though this case has no other stigmata of taberous sclerosis complex, it may pathogenetically represents the so-called "formes frustes" of that syndrome. Angiomyolipomas is occasionally diagnosed as malignant by pathologists because of its mimicking histologic features. Since angiomyolipoma is a benign tumor, the treatment differs greatly from that of renal cell carcinoma. More precise examinations will enable us to do non-operative observation or limited surgery with preservation of the renal parenchyma.