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[结节性硬化症患者肾血管平滑肌脂肪瘤的临床特点与治疗]

[The clinical aspects and treatment of renal angiolipomas in patients with tuberous sclerosis].

作者信息

van Baal J G, Fleury P, Brummelkamp W H

机构信息

Ziekenhuis St. Franciscus, afd. Chirurgie, Roosendaal.

出版信息

Ned Tijdschr Geneeskd. 1990 Aug 11;134(32):1556-8.

PMID:2392178
Abstract

The incidence of multiple and bilateral renal angiomyolipomas in tuberous sclerosis patients is 40-80%. These benign abundantly vascularised tumours are almost always asymptomatic. Most of the symptomatic renal angiomyolipomas measure more than 4 cm. These lesions are attended by a high risk of spontaneous rupture and massive haemorrhage. In our series of 23 tuberous sclerosis patients with renal angiomyolipomas 4 became symptomatic. Three of them were successfully treated wtih transcatheter selective embolization. Preventive embolization of renal angiomyolipomas appears indicated if these measure more than 4 cm. A fifth patient became symptomatic before the diagnosis of tuberous sclerosis was made. She had a forme fruste. She was also successfully treated by the same method.

摘要

结节性硬化症患者中多发及双侧肾血管平滑肌脂肪瘤的发生率为40%-80%。这些良性的、血管丰富的肿瘤几乎总是无症状的。大多数有症状的肾血管平滑肌脂肪瘤直径超过4厘米。这些病变有很高的自发破裂和大出血风险。在我们的23例患有肾血管平滑肌脂肪瘤的结节性硬化症患者系列中,4例出现了症状。其中3例通过经导管选择性栓塞成功治疗。如果肾血管平滑肌脂肪瘤直径超过4厘米,似乎有必要进行预防性栓塞。第五例患者在结节性硬化症诊断之前就出现了症状。她有顿挫型。她也通过同样的方法成功治疗。

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