Yasukawa S, Aoshi H, Kyoku I, Uekado Y, Ohkawa T
Department of Urology, Wakayama Medical College.
Hinyokika Kiyo. 1989 Dec;35(12):2135-8.
Renal angiomyolipoma associated with tuberous sclerosis is well known. On the other hand, few cases of renal cell carcinoma in connection with tuberous sclerosis have been reported. We report a patient with tuberous sclerosis whose kidney was involved with renal cell carcinoma. A 18-year-old woman was first admitted in August 1987 for evaluation of left renal tumor. Diagnosis of tuberous sclerosis was made when she was 11 years old on the basis of mental retardation, papules on her face, seizures, white leaf-shaped macules and periventricular calcifications. Computerized tomographic scan demonstrated a large mass arising from the left kidney and small masses in the right kidney. Angiography confirmed bilateral hypervascular renal tumors. On these bases, a clinical diagnosis of bilateral renal angiomyolipomas was made and surgical treatment of the left kidney was recommended because of its large size. However, her parents did not permit treatment until March, 1988. Finally, left nephrectomy of 4,750 g was performed and histological examination revealed renal cell carcinoma with clusters of spindle cells. In the literature available to us, we found twelve reports of malignant renal tumors associated with tuberous sclerosis including five renal cell carcinomas in Japan.
肾血管平滑肌脂肪瘤与结节性硬化症相关是众所周知的。另一方面，与结节性硬化症相关的肾细胞癌病例报道较少。我们报告一例患有结节性硬化症且肾脏发生肾细胞癌的患者。一名18岁女性于1987年8月首次入院，以评估左肾肿瘤。她11岁时因智力发育迟缓、面部丘疹、癫痫发作、白色树叶状斑和脑室周围钙化被诊断为结节性硬化症。计算机断层扫描显示左肾有一个大肿块，右肾有小肿块。血管造影证实双侧肾肿瘤血管丰富。基于这些，临床诊断为双侧肾血管平滑肌脂肪瘤，因左肾肿块较大，建议行左肾手术治疗。然而，她的父母直到1988年3月才同意治疗。最终，进行了左肾切除术，切除的左肾重4750克，组织学检查显示为肾细胞癌，伴有梭形细胞簇。在我们所能查阅的文献中，我们发现有12篇关于与结节性硬化症相关的恶性肾肿瘤的报道，其中包括日本的5例肾细胞癌。
