Pleuropulmonary blastoma is an uncommon malignant lung tumor observed in ch
ildren. Outcome is often unfavorable. Two boys and two girls, mean age 4.5
years, were admitted for nonspecific respiratory signs. Oriented by radiolo
gy findings, the diagnosis of pleuropulmonary blastoma was confirmed at pat
hology examination of a pneumonectomy specimen. Three of the children were
given postoperative adjuvant chemotherapy. There were three deaths and one
child,vas lost to follow-up.
We discuss the clinical features of pleuropulmonary blastoma. No optimal tr
eatment has been defined for this often fatal tumor.