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与结节性硬化症相关的巨大双侧肾血管平滑肌脂肪瘤。

Giant bilateral renal angiomyolipomas associated with tuberous sclerosis.

作者信息

Ciancio S J, Vira M, Simon M A, Lerner S P, Schulam P G

机构信息

Scott Department of Urology, Baylor College of Medicine and Methodist Hospital, Houston, Texas, USA

出版信息

Urology. 2001 Mar;57(3):554. doi: 10.1016/s0090-4295(00)01051-7.

Abstract

Renal angiomyolipomas in patients with tuberous sclerosis can be associated with significant morbidity, mostly related to complications from bleeding. We describe a patient with tuberous sclerosis and massive bilateral renal angiomyolipomas (total tumor burden 5500 g) who presented with acute right renal hemorrhage. She was treated with right renal artery embolization followed immediately by right nephrectomy and left partial nephrectomy. The patient had a creatinine nadir of 1.3 mg/dL postoperatively. We demonstrate that nephron-sparing surgery is feasible, even in the setting of very large angiomyolipomas, such as the one presented here, currently the largest such tumor by weight reported.

摘要

结节性硬化症患者的肾血管平滑肌脂肪瘤可伴有严重的发病情况,主要与出血并发症相关。我们描述了一名患有结节性硬化症和双侧巨大肾血管平滑肌脂肪瘤(肿瘤总重量5500克)的患者,该患者出现急性右肾出血。她接受了右肾动脉栓塞治疗,随后立即进行了右肾切除术和左肾部分切除术。患者术后肌酐最低点为1.3mg/dL。我们证明,即使是对于非常大的血管平滑肌脂肪瘤,如本文所呈现的目前按重量报告的最大此类肿瘤,保留肾单位手术也是可行的。

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