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先天性肺囊肿内发生的横纹肌肉瘤:三例报告

Rhabdomyosarcoma arising within congenital pulmonary cysts: report of three cases.

作者信息

Murphy J J, Blair G K, Fraser G C, Ashmore P G, LeBlanc J G, Sett S S, Rogers P, Magee J F, Taylor G P, Dimmick J

机构信息

Division of Pediatric General Surgery, British Columbia Children's Hospital, Vancouver, Canada.

出版信息

J Pediatr Surg. 1992 Oct;27(10):1364-7. doi: 10.1016/0022-3468(92)90299-m.

Abstract

Over the past 9 months, three cases of primary pulmonary rhabdomyosarcoma have been treated at British Columbia Children's Hospital. Two patients (aged 24 and 37 months) presented with spontaneous pneumothoraces and had cystic changes in the affected lung on chest radiograph. The third patient (aged 42 months) was evaluated for chronic cough, fever, and failure to thrive. Chest x-ray showed a large mass in the left lower lobe as well as mediastinal adenopathy. All three of these lesions originated within congenital lung cysts, one a peripheral bronchogenic cyst and the others cystic adenomatoid malformations. This report suggests that there is a significant risk for the development of rhabdomyosarcoma within malformed pulmonary tissue.

摘要

在过去9个月里,不列颠哥伦比亚省儿童医院收治了3例原发性肺横纹肌肉瘤患者。两名患者(年龄分别为24个月和37个月)表现为自发性气胸,胸部X光片显示患侧肺有囊性改变。第三名患者(42个月大)因慢性咳嗽、发热和发育不良接受评估。胸部X光显示左下叶有一个大肿块以及纵隔淋巴结肿大。所有这三个病变均起源于先天性肺囊肿,其中一个是外周支气管源性囊肿,其他是囊性腺瘤样畸形。本报告表明,在畸形肺组织内发生横纹肌肉瘤的风险很高。

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