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病例报告:结节性硬化症中的肾周血管平滑肌脂肪瘤

Case report: perinephric angiomyolipomas in tuberous sclerosis.

作者信息

Peh W C, Lim B H, Tam P C

机构信息

Department of Diagnostic Radiology, University of Hong Kong, Queen Mary Hospital.

出版信息

Br J Radiol. 1994 Oct;67(802):1026-9. doi: 10.1259/0007-1285-67-802-1026.

Abstract

Bilateral large perinephric angiomyolipomas in a young Chinese woman with tuberous sclerosis are reported. Computed tomography (CT) demonstrated large fatty masses, containing abnormally dilated blood vessels and muscle strands, within the Gerota's fascia bilaterally. There were multiple foci of involvement in both kidneys. The diagnosis was confirmed on histological examination of the excised left perinephric angiomyolipoma and nephrectomy specimens. The patient subsequently developed bilateral pneumothoraces which were surgically treated. High resolution CT showed extensive thin-walled cysts in both lungs.

摘要

报告了一名患有结节性硬化症的年轻中国女性双侧巨大肾周血管平滑肌脂肪瘤。计算机断层扫描(CT)显示双侧肾周筋膜内有巨大脂肪肿块,包含异常扩张的血管和肌束。双肾均有多个受累病灶。通过对切除的左侧肾周血管平滑肌脂肪瘤和肾切除标本进行组织学检查确诊。患者随后出现双侧气胸并接受了手术治疗。高分辨率CT显示双肺有广泛的薄壁囊肿。

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