Nakamura Y, Murayama K, Katsumi T, Watanabe K, Kishitani M, Ohkawa M
Department of Urology, Kanazawa National Hospital.
Hinyokika Kiyo. 1994 Aug;40(8):703-6.
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.
尽管结节性硬化症与肾血管平滑肌脂肪瘤并存已广为人知，但结节性硬化症合并肾细胞癌和肾血管平滑肌脂肪瘤的情况极为罕见。本文报道了1例结节性硬化症患者并发双侧肾血管平滑肌脂肪瘤和左肾细胞癌的病例。一名40岁男性因左侧腰部肿块被转诊至我院进一步评估和治疗。根据皮脂腺瘤、癫痫发作、智力发育迟缓及脑室周围钙化诊断为结节性硬化症。增强CT扫描显示左肾上极和中部有不规则强化肿块，双侧肾脏有多个小的低密度结节。选择性左肾血管造影显示左肾上极和中部有高血运区。根据所获结果，临床诊断为左肾细胞癌合并双侧肾血管平滑肌脂肪瘤。行左肾根治性切除术。组织病理学检查显示为肾细胞癌和多个肾血管平滑肌脂肪瘤结节。本文讨论了诊断要点，尤其是通过影像学特征对肾血管平滑肌脂肪瘤和肾细胞癌进行鉴别诊断。
