Clinical characteristics and surgical treatment of congenital cystic adenomatoid malformation in adults: the largest cohort of 46 patients

BACKGROUND: Congenital cystic adenomatoid malformation (CCAM) is a rare congenital malformation of the lungs, however it lacks a summary of pathognomonic clinical and imaging features in adults. Our study aims to evaluate clinical characteristics and surgical treatment in the largest case series of adult CCAM. METHODS: The records of 46 adult patients with CCAM admitted to West China Hospital between February 2009 and March 2019 were reviewed. All patients accepted the surgery and get fully recovered. Data were collected and analyzed regarding patient demographics, medical history, preoperative investigations, intraoperative findings, and postoperative outcomes. RESULTS: The records of 22 men and 24 women were examined. The main systemic and respiratory symptoms included fever, productive cough, hemoptysis, and chest pain. Twenty lesions were found in the right pulmonary lobes and 26 in the left lobes. All CCAM lesions were successfully resected by surgery (35 patients had lobectomies, and the remaining 11 patients underwent wedge resections). Twenty-nine patients underwent video-assisted thoracic surgery (VATS), while 17 patients received posterolateral thoracotomy (PLT). The pathological analysis of surgical specimens revealed 26 cases of pure CCAM lesions and 20 cases of CCAM mixed with other diseases. More than 10% of patients had coexisting pre-malignant or malignant lung lesions. Four patients experienced postoperative complications. No intraoperative and postoperative deaths occurred. CONCLUSIONS: Surgical resection remains the preferred approach for adults with CCAM and has satisfied outcomes. Clinicians should be aware of possible coexisting infections and malignancies.