Congenital pulmonary airway malformation of lung in fetus：a clinicopathological analysis
10.3760/cma.j.issn.0529-5807.2015.04.012
- VernacularTitle:胎儿先天性肺气道畸形的临床病理分析
- Author:
Yingnan WANG
1
;
Yiqun GU
;
Xiaobo ZHANG
;
Aichun WANG
;
Junling XIE
;
Lijuan LU
;
Yunfei SUN
Author Information
1. 100080,北京市海淀区妇幼保健院病理科
- Keywords:
Pulmonary airway malformation;
Lung;
Fetus;
Autopsy;
Immunohistochemistry
- From:
Chinese Journal of Pathology
2015;(4):266-269
- CountryChina
- Language:Chinese
-
Abstract:
Objective To study the etiology, pathogenesis, clinicopathologic characteristics, prognosis and treatment of congenital pulmonary airway malformation (CPAM).Methods Eighteen cases of CPAM were enrolled into the study.The clinical history, autopsy findings and immunohistochemical results were evaluated, with review of literature.The pathogenetic mechanism, pathologic features and differential diagnosis of CPAM were studied.Results Histologic examination showed that 2 cases were classified as Stocker typeⅠ, 12 cases as type Ⅱ, and 4 cases as type Ⅲ.The lesion was unilateral and involved single lobe in 13 cases.The remaining 5 cases had bilateral diseases.Of the 18 cases studied, 12 cases showed single organ involvement and 6 cases had malformations affecting multiple organs.The associated malformations included cardiac anomalies ( 4 cases ) , polycystic kidney with gastrointestinal atresia (1 case) and nuchal cystic hygroma with hydrothorax (1 case).Conclusions CPAM is a rare pulmonary disorder.The etiology of this non-neoplastic condition is unknown.Imaging analysis is a valuable tool to suggest CPAM, while definite diagnosis requires pathologic examination.The overall prognosis is determined by the presence of associated malformations, fetal hydrops and pulmonary hypoplasia.
