CHILDHOOD PLEUROPULMONARY BLASTOMA - CAUTION AGAINST NONOPERATIVE MANAGEMENT OF CONGENITAL LUNG CYSTS

Pulmonary blastoma is a rare and aggressive malignant tumor that affec ts children and adults. Recently a 3-year-old boy with a 2-year histor y of bilateral unilocular pulmonary cysts was transferred for evaluati on of a cough and high spiking fever. A chest radiogram showed left pu lmonary consolidation with pleural effusion, but thoracentesis was uns uccessful. Computerized tomography (CT) was suggestive of a pulmonary abscess, but CT-guided drainage did not yield any purulent fluid. Perc utaneous biopsies were performed, and the cytology showed malignant ce lls. During thoracotomy, a large tumor involving the left lower lobe a nd pleural space was found, and a biopsy was performed. A frozen secti on showed blastemal and mesenchymal components devoid of neoplastic ep ithelium, consistent with the pleural variant of pulmonary blastoma. A left lower lobectomy, with tumor decortication of the pleural space, achieved total gross tumor removal. The child received aggressive mult iagent chemotherapy, and midway through it he underwent elective excis ion of the opposite lung cyst. It has been 17 months since the lobecto my; he is off chemotherapy and has no evidence of disease. A review of the literature showed that a large number of pediatric pulmonary blas tomas are associated with cystic lung disease. Because total tumor rem oval offers the only chance of a good long-term outcome, surgical exci sion or close follow-up of pulmonary cysts in children is strongly rec ommended. Copyright (C) 1996 by W.B. Saunders Company.