先天性肺囊性腺瘤样畸形43例回顾性分析并文献复习

摘要

Objective:To investigate the pathological classification and prognosis of congenital pulmonary cystic adenomatoid malformation (CCAM)in children.Methods:The pathological diagnosis,clinical manifestation and ima-ging changes of 43 children with CCAM treated at Xi＇an Children＇s Hospital from 2001 to 2017 were analyzed retro-spectively,and the relevant literature was reviewed.Results:Of the 43 CCAM children,19 were CCAM type I,21 were CCAM typeⅡ,2 were CCAM typeⅣand 1 case of CCAM type I and CCAM typeⅡoverlapping were accompanied by multifocal mucinous bronchioloalveolar carcinoma (BAC).Conclusion:CCAM in children has the possibility of malignant tumor or transformation to malignant tumor.This malignant transformation can occur in very early stage of the infancy,and its pathological type is helpful to judge the prognosis of CCAM in children.%目的:探讨先天性肺囊性腺瘤样畸形(CCAM)患儿的病理分型及预后.方法:回顾分析2001年至2017年在西安市儿童医院就诊的43例CCAM患儿的病理诊断及临床表现、影像学改变,并复习相关文献.结果:43例CCAM患儿中,19例为CCAM Ⅰ型,21例为CCAM Ⅱ型,2例为CCAM Ⅳ型,1例CCAM Ⅰ型和CCAM Ⅱ型重叠伴有黏液性细支气管肺泡癌.结论:小儿CCAM有伴发恶性肿瘤或向恶性肿瘤转变的可能,这种恶性转化可以发生在婴儿期早期,其病理学分型有助于判断CCAM患儿的预后.