Management of pulmonary blastoma in children.

Pulmonary blastoma is a rare neoplasm, accounting for 0.5 per cent of all primary malignant pulmonary tumors. The clinical features, radiologic findings, and management of five children with pulmonary blastoma are described. The female/male ratio was 4:1. Mean age at presentation was 34 months, with a mean duration of symptoms of 1.8 months. Fever and cough were the most common symptoms. Opacification of the right lung field with mediastinal shift to the left was the predominant roentgenologic feature. The tumor involved the right chest in all patients. Thoracotomy was performed in all patients to establish the diagnosis. Two patients underwent complete resection at the initial operation, an incomplete resection was performed in one patient. Delayed complete resection was performed in another patient. All the patients received combination chemotherapy and one patient also received radiotherapy for primary disease. Two patients with histologic and immunohistochemical evidence of striated muscle differentiation were treated as rhabdomyosarcomas. Both these patients have survived for 24 and 67 months after diagnosis. The tumor recurred in three patients. Recurrence was treated with a combination of surgery, chemotherapy, and radiotherapy. Overall disease-free survival rate was 40 per cent. Long-term follow-up and additional patients are required to provide prognostic information.