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Congenital Cystic Adenomatoid lung Malformation: what is the risk of degeneration?

A. Victor, JC. Dubus, C. Fernandes, F. Nicolas, G. Gorincour, JM. Guys, P. de Lagausie

Department of Pediatric Surgery, La Timone Children Hospital, Marseille, France



Introduction: The question of the surgical or conservatory treatment of adenomatoid cystic lung malformations (CCAM) still has no precise answer. The aim of this study is to evaluate the risk of degeneration of a pre-existent CCAM lesion.

Material and methods: We made a very exhaustive literature review. The search engines were Pubmed and Scorpus with the following key-words: broncho-alveolar, carcinoma, rhabdomyosarcoma, malignant transformation, pleuropneumoblastoma, childhood, congenital cystic adenomatoid malformation, cystic lung disease. The articles that we found were read (surgeon and anatomopathologist) in order to validate the characteristics of the described lesions and to keep only the information that concerned the CCAM degeneration. The bonchogenic cystic were excluded. The lesions that didn’t have a formal anatomopathologic diagnosis were classified apart.

Results: The review of 33 articles allowed the selection of 42 cases of malignant transformation of CCAM. The different types of cancer were: bronchial alveolar carcinomas in 22 cases (52,4%), rhabdomyosarcoma in 13 cases (31%), pleuropneumoblastoma in 6 cases (14,3%) and a myxosarcoma in a case (2,4%). If we consider an incidence of 1/25000 to 1/35000 living births, the risk of degeneration in bronchial alveolar carcinoma is evaluated at 1%.

Discussion: The risk of malignant transformation of the lung cystic malformations, even if it is rare, it has been confirmed. The bronchial alveolar carcinoma affects the young people with type I CCAM. Concerning the risk of rhabdomyosarcoma, it is more important in case of CCAM than in the standard population (lung rhabdomyosarcoma is extremly rare!). Finally, other malignant transformations are possible. Concerning the pleuropneumoblastoma, it can be considered as an association to a pre-existent cyst or as a cystic form of the tumor.

Conclusion: These evolutive risks prove for us that early surgery is necessary with exeresis of the primary malformative disease.


Key words: adenomatoid cystic lung malformation, bronchial alveolar carcinoma, rhabdomyosarcoma, pleuropneumoblastoma