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婴儿胸壁错构瘤

Hamartomas of the chest wall in infants.

作者信息

Dounies R, Chwals W J, Lally K P, Isaacs H, Senac M O, Hanson B A, Mahour G H, Sherman N J

机构信息

Department of Pediatric Surgery, Children's Hospital of Los Angeles, University of Southern California.

出版信息

Ann Thorac Surg. 1994 Apr;57(4):868-75. doi: 10.1016/0003-4975(94)90192-9.

Abstract

Chest wall hamartomas in infancy are rare lesions with distinct clinical, radiologic, and pathologic characteristics. Four cases treated at Children's Hospital of Los Angeles are presented and previously reported cases are reviewed. Chest wall hamartomas arise antenatally and present as hard, immobile masses, which may cause respiratory insufficiency. An extrapleural mass arising from the ribs can be seen radiographically. Histologically, these lesions are hypercellular and consist of a disorganized array of mesenchymal tissues endogenous to the chest wall. Rapid growth may occur, but usually is self-limited. Chest wall hamartomas are usually benign. This series includes the malignant transformation of one of these lesions. En bloc resection is curative, but the large residual chest wall defect frequently results in scoliosis.

摘要

婴儿期胸壁错构瘤是一种罕见的病变,具有独特的临床、放射学和病理学特征。本文报告了洛杉矶儿童医院治疗的4例病例,并对既往报道的病例进行了回顾。胸壁错构瘤在产前发生,表现为坚硬、固定的肿块,可导致呼吸功能不全。影像学检查可见肋骨起源的胸膜外肿块。组织学上,这些病变细胞丰富,由胸壁内源性间叶组织的无序排列组成。可能会出现快速生长,但通常为自限性。胸壁错构瘤通常为良性。本系列包括其中1例病变的恶性转化。整块切除可治愈,但巨大的胸壁残余缺损常导致脊柱侧凸。

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