- 作者：Ahmed Nasr ; Sharifa Himidan ; Aimee C. Pastor ; Glenn Taylor ; Peter C.W. Kim
- 关键词：Congenital cystic adenomatoid malformation ; Pulmonary blastoma ; Cancer risk
- 刊名：Journal of Pediatric Surgery
- 出版年：2010
- 出版时间：June 2010
- 年：2010
- 卷：45
- 期：6
- 页码：1086-1089
- 全文大小：214 K
BackgroundThe relationship between congenital cystic adenomatoid malformation (CCAM) and pleuropulmonary blastoma (PPB), whether causal, correlational, or coincidental, remains controversial. There is a lack of consensus as to the optimal treatment of patients with asymptomatic CCAM.Method
We reviewed all cases of CCAM and PPB seen at our institution from 1999 to 2008. Institutional Research Ethics Board approval was obtained. The incidence of CCAM and PPB, respectively, was calculated based on birth numbers during the study period.
Results
Seventy-four CCAMs were resected over the study period in 129 children diagnosed with CCAM. Five PPBs were diagnosed during the study period. Three of the 5 PPB cases were initially diagnosed as CCAMs. These PPBs were not clinically or radiologically distinguishable from CCAMs. In our referral area, the incidence of CCAM was 1 in 12,000; and the incidence of PPB was 1 in 250,000 live births. The mortality rate for PPB in this cohort was 20 % .
Conclusion
Asymptomatic cystic lung malformations represent a therapeutic dilemma. In this cohort, the incidence of PPB among apparently benign lung lesions was 4 % . No clinical or radiological markers differentiated benign CCAMs from PPBs. Our experience provides further justification for resection of all CCAMs. This should be discussed with parents until CCAMs and PPBs can be clearly distinguished preoperatively.