Primary epithelial lung malignancies in the pediatric population.

BACKGROUND: Primary epithelial lung malignancies are rare in childhood and adolescence. We reviewed the Memorial Sloan-Kettering Cancer Center experience with these tumors to better understand their histology, time to diagnosis, treatment, and outcome.

PROCEDURE: A retrospective review was performed on all patients 21 years of age or younger at diagnosis, treated for primary epithelial lung malignancies at Memorial Sloan-Kettering Cancer Center between 1980 and 2001.

RESULTS: We identified 11 patients with primary epithelial lung malignancy. The median age at diagnosis was 19 (range: 12-21) years. The most common radiographic abnormality was a mass (55%) on chest imaging. Seven patients (64%) were initially diagnosed as having pneumonia which contributed to a delay in diagnosis. Final pathologic diagnoses included adenocarcinoma (four), carcinoid tumor (three typical, one atypical), basaloid carcinoma (two), and mucoepidermoid carcinoma (one). A majority of patients presented with advanced disease (two stage III, four stage IV). Patients with localized disease were treated with surgical resection and all but one remains disease free with a median follow-up of 60 months (range: 13-286). Patients with either advanced locoregional or distant metastatic disease were treated with multimodal therapy and a majority had rapid progression of disease.

CONCLUSIONS: When children and adolescents present with primary epithelial lung malignancy a majority will have advanced disease and experience a delay in diagnosis. The histologic types of tumors encountered are similar to lung tumors occurring in adults, although the frequency of the various types differs. Carcinoid tumors are more frequent, and less common subtypes of bronchogenic carcinoma are also more prevalent in the pediatric age group. Similar to the adult population, the prognosis of these tumors is dependent on histology and stage. Patients with carcinoid tumors seem to have the best prognosis, followed by adenocarcinoma. The highly aggressive basaloid carcinoma has the worst.