Primary malignant pulmonary tumors in children: a review of the national cancer data base

Abstract

Background: The purpose is to delineate the clinical and pathological characteristics of rare primary malignant pulmonary tumors in children.

Methods: Utilizing the National Cancer Data Base (NCDB), we analyzed all children (≤ 18 years) with a primary malignant pulmonary tumor from 1998 to 2011 to identify factors associated with better survival.

Results: Of 211 children identified, the most common histology was carcinoid tumor (n=133, 63%) followed by mucoepidermoid carcinoma (MEC) (n=37, 18%), squamous cell carcinoma (SCC) (n=19, 9%), adenocarcinoma (n=16, 8%), bronchoalveolar carcinoma (BAC) (n=4, 2%), and small cell carcinoma (SCLC) (n=2, <1%). Factors that significantly affected survival include histology, race, tumor size, lymph node status, and extent of surgery. Patients with MEC and carcinoid tumors had a better overall survival compared to patients with other histologies (p<0.0001). The 5-year overall survival for MEC and carcinoid tumors was 100% and 95% (95% CI 87-98), respectively, versus 50% (95%CI 1-91) for BAC, 28% (95%CI 9-52) for SCC, and 26% (95%CI 5-55) for adenocarcinoma.

Conclusion: The majority of pediatric patients with a primary malignant pulmonary tumor present with carcinoid tumor or MEC and have an excellent prognosis. Lung cancers which are common in adults, but rare in children, have a worse prognosis.

Keywords: National Cancer Database (NCDB); Pediatric; Population-based study; Primary lung cancer.