Pleuropulmonary blastoma: an aggressive intrathoracic neoplasm of childhood.

A case of a 4-year-old girl with pleuropulmonary blastoma is reported. Surgical resection of the tumor was performed and histologic examination revealed pleuropulmonary blastoma with rhabdomyosarcomatous differentiation. Postoperative chemotherapy was administered and 3 weeks after initiation of treatment protocol a second site of lesion in the retroperitoneum was revealed with extension to the mediastinum, which shared similar mesenchymal neoplastic characteristics to the previously diagnosed primary lesion. The girl died 4 1/2 months after initial evidence of disease because of brain metastasis, indicating a very aggressive neoplasm unresponsive to treatment.