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先天性囊性腺瘤样畸形

Congenital cystic adenomatoid malformation.

作者信息

Rosado-de-Christenson M L, Stocker J T

机构信息

Department of Radiologic Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000.

出版信息

Radiographics. 1991 Sep;11(5):865-86. doi: 10.1148/radiographics.11.5.1947321.

Abstract

Congenital cystic adenomatoid malformation of the lung is a rare lesion that typically manifests as neonatal respiratory distress secondary to progressive expansion of the affected lung. Three distinct types have been described based on the size of the cysts and the microscopic appearance. Type I lesions are characterized by large cysts of varying sizes (measuring more than 2 cm in diameter), type II lesions typically contain cysts of a more uniform size (not exceeding 2 cm in diameter), and type III lesions contain microscopic cysts. Radiologic findings include single or multiple large cysts, multiple small cysts of uniform size, and solid-appearing masses. The treatment of choice is excision of the affected lobe. The prognosis is favorable in the absence of pulmonary hypoplasia, fetal hydrops, or associated congenital anomalies.

摘要

先天性肺囊性腺瘤样畸形是一种罕见的病变,通常表现为由于患侧肺进行性扩张继发的新生儿呼吸窘迫。根据囊肿大小和显微镜下表现,已描述了三种不同类型。I型病变的特征是大小不一的大囊肿(直径超过2厘米),II型病变通常含有大小更均匀的囊肿(直径不超过2厘米),III型病变含有微小囊肿。影像学表现包括单个或多个大囊肿、多个大小均匀的小囊肿以及实性肿块。首选的治疗方法是切除患侧肺叶。在没有肺发育不全、胎儿水肿或相关先天性异常的情况下,预后良好。

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