Primary lung tumors in children and adolescents: a 90-year experience

Primary lung tumors in children are rare. A wide range of histopathologic tumor types occurs. The incidence of these lesions and their outcomes are still largely unknown. This study aims to determine the incidence of different primary lung tumors in children and to contribute data leading to the development of evidence-based treatment models.

A single institution retrospective review was performed with institutional review board approval. Patients were included if they had primary, nonhematologic lung tumors. Simple squamous papillomas subjected to endoscopic biopsy and not resected, and vascular lesions associated with multisystem lesions, such as hereditary hemorrhagic telangiectasia, were excluded. Medical records and pathologic material for patients from 1918 to 2008 were reviewed.

Forty patients were identified (23 boys, 17 girls) with a mean age of 9.6 years (range, 3 months to 19 years). Fourteen distinct histopathologic tumor types were identified. The most common tumor types were carcinoid (8), inflammatory myofibroblastic tumor (7), and pleuropulmonary blastoma (6). Rare pediatric lung tumors including small cell carcinoma, adenocarcinoma, and pulmonary capillary hemangiomatosis were also seen. The mortality rate was 17.5% (7) in our series. Chemotherapy was used in 23% (9) and radiation in 20% (8) of the patients. Of the 33 survivors, 28 had follow-up with a median duration of 29.5 months (mean, 63.2 months; range, 1-471 months).

Primary lung tumors in children are rare and histopathologically diverse. The tumor spectrum involves many types not seen in adults, and unlike adults, patients rarely have a history of exposure to external predisposing factors. Although complete resection remains the standard for treatment of most tumors, addition of adjuvant therapy is dependent on both tumor stage and histopathologic type.