Rare Tumours of the Lung: A Single Centre Experience

Introduction: A rare tumour of the lung (RTL) is defined as neoplasm whose incidence is lower than 2% of all pulmonary tumours. Such group includes different benign and malignant lesions with variable occurrence rate. Aim of this study was to retrospectively analyze a single centre surgical experience and com- pare it with the literature. Patients and Methods: From February 2002 to April 2009 twenty-four patients underwent resection for RTL. Different procedures were utilized, including operative rigid bronchoscopy, wedge resection, lobectomy, pneumonectomy, and bronchoplastic procedures. All patients entered a follow-up program (median 22, range1-79 months). Results: According to the 2004 WHO classification 6 patients had benign (Group A) and 18 malignant (Group B) RTL. Group A: endobronchial hamartoma (n=1) endobronchial lipoma (n=1), endobronchial fibroepithelial polyp (n=1), benign fibrous hystiocitoma (n=1) and sclerosing hemangioma (n=2). Group B: pulmonary blastoma (B1, n=1), mucoepidermoid carcinoma (B2, n=1), adenoid cystic carcinoma (B3, n=3), inflammatory myofi- broblastic tumour (B4, n=1), pulmonary synovial sarcoma (B5, n=3), dendritic follicular cells sarcoma (B6, n=1), pleomorphic carcinoma (B7, n=2) spindle cell carcinoma (B8, n=2), and carcinosarcoma (B9, n=4). Symptoms were present mostly in centrally- located neoplasms (10/24, n=3 Group A and n=7 Group B). Two patients were lost at follow-up. Three patients with B9 died of the disease (median 14 months, range 4-15), one B8 immunocompromised patient had progressive disease after 3 months, the remaining are present alive without evidence of disease. Conclusions: Our results substantially reflect the literature data. A multicentre study would add to the knowledge of this heterogeneous group of tumours.