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双侧第一肋骨巨大纤维结构不良:纵隔及胸廓出口受压

Giant bilateral fibrous dysplasia of first ribs: compression of mediastinum and thoracic outlet.

作者信息

Thomas de Montpréville V, Dulmet E, Ponlot R, Dartevelle P

机构信息

Dept of Pathology, Marie Lannelongue Surgical Centre (Université Paris-Sud), Le Plessis-Robinson, France.

出版信息

Eur Respir J. 1995 Jun;8(6):1028-9.

PMID:7589367
Abstract

A 43 year old woman presented with two large bilateral tumours of the first ribs that compressed the right subclavian vessels, trachea and oesophagus and led to right arm oedema, severe dyspnoea and dysphagia. The resected tumours showed typical histological features of fibrous dysplasia without malignant transformation. The right tumour weighed 1.5 kg and measured 17 x 13 x 10 cm. This report demonstrates that major surgery is still possible for resection of such exceptional giant compressive lesions, since fibrous dysplasia is a benign and non-infiltrative tumour.

摘要

一名43岁女性因双侧第一肋骨出现两个大肿瘤前来就诊,这些肿瘤压迫右锁骨下血管、气管和食管,导致右臂水肿、严重呼吸困难和吞咽困难。切除的肿瘤显示出骨纤维发育不良的典型组织学特征,无恶性转化。右侧肿瘤重1.5千克,尺寸为17×13×10厘米。本报告表明,对于切除此类罕见的巨大压迫性病变,大手术仍然可行,因为骨纤维发育不良是一种良性且无浸润性的肿瘤。

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