Case report: pulmonary blastoma in children--response to chemotherapy

Pulmonary blastoma (PB) is a rare primary malignancy of the lung, with about 54 cases reported in children. The tumor consists of mesenchymal and epithelial components resembling the fetal lung. It has been treated primarily with surgery and the effect of combination chemotherapy has not been systematically investigated. A 15-year-old girl with PB with metastases to bone and regional lymph node, and high levels of alphafetoprotein, is reported. A preoperative combination chemotherapy consisting of cisplatinum, etoposide alternating with iphosphamide with mesna, vincristine and epirubicine resulted in an objective response that permitted subsequent safe surgical excision of the primary tumor. This intensive combination chemotherapy should be tested in the management of advanced PB in children, as initial therapy as well as an adjuvant to surgery.