自然破裂1例を含む腎血管筋脂肪腫の2例

Abstract

Two cases of renal angiomyolipoma with tuberous sclerosis are reported. The first was a 42-year-old housewife with complaints of left flank pain, palpable mass at the same region and in a preshock state. Ultrasonogram of the left kidney demonstrated a large echogenic mass with fluid. Preoperative diagnosis was renal angiomyolipoma with spontaneous rupture. Emergency nephrectomy and lymphadenectomy were performed. The histopathological diagnosis was renal angiomyolipoma with nodal involvements which reflects the multicentricity of the disorder. The second was a 44-year-old housewife with dull right flank pain. CT scan showed a mass in the upper part of the kidney which had an attenuation value of -60 HU. Adrenal tumor could not be distinguished from renal tumor by selective renal angiogram. Nephrectomy was performed and histopathological diagnosis was renal angiomyolipoma. Both cases remain well. We reviewed 32 cases of renal angiomyolipoma with spontaneous rupture in the Japanese literature with respect to age, affected side, presence or absence of tuberous sclerosis, preoperative diagnosis and treatment. Previous diagnoses were done with plain film, excretory urogram and angiogram. Preoperative diagnosis of angiomyolipoma was difficult or impossible, and operative procedure of choice was nephrectomy. Now CT and ultrasonogram are the specific tools in the diagnosis of angiomyolipoma. Partial nephrectomy, enucleation or observation is selected because renal angiomyolipoma is a benign tumor.