Amano M, Okunobo T, Kawahara H, Ueda H, Tanaka H
Hinyokika Kiyo. 1984 Dec;30(12):1813-25.
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.
报告了两例合并结节性硬化症的肾血管平滑肌脂肪瘤。第一例是一位42岁的家庭主妇,主诉左侧胁腹疼痛,同一区域可触及肿块,处于休克前期状态。左肾超声检查显示一个大的高回声肿块伴液性暗区。术前诊断为肾血管平滑肌脂肪瘤伴自发性破裂。行急诊肾切除术和淋巴结切除术。组织病理学诊断为肾血管平滑肌脂肪瘤伴淋巴结受累,这反映了该疾病的多中心性。第二例是一位44岁的家庭主妇,右侧胁腹隐痛。CT扫描显示肾上部有一肿块,衰减值为-60 HU。选择性肾血管造影无法区分肾上腺肿瘤和肾肿瘤。行肾切除术,组织病理学诊断为肾血管平滑肌脂肪瘤。两例患者目前情况良好。我们回顾了日本文献中32例肾血管平滑肌脂肪瘤伴自发性破裂的病例,涉及年龄、患侧、是否存在结节性硬化症、术前诊断和治疗情况。既往诊断采用平片、排泄性尿路造影和血管造影。肾血管平滑肌脂肪瘤的术前诊断困难或无法进行,首选的手术方式是肾切除术。现在CT和超声检查是诊断肾血管平滑肌脂肪瘤的特异性工具。由于肾血管平滑肌脂肪瘤是良性肿瘤,可选择部分肾切除术、肿瘤剜除术或观察。