Pleuropulmonary blastoma: case report

ABSTRACT

Pleuropulmonary blastoma [PPB] is a rare and aggressive tumor that is emerging as a distinct entity of childhood disease .It is characterized by mesenchymal elements [including undifferentiated blastoma and often cartilaginous, rhabdomyoblastic, or fibroblastic differentiation] and epithelium-lined spaces. PPB may be exclusively cystic [type I], solid [type III] or both solid and cystic [type II]. A 5-month-old boy presented with a history of fever and respiratory distress. Chest radiograph and subsequent CT scan showed a large soft-tissue density occupying the left chest cavity. Radical resection of the mass was achieved by lobectomy. Histologic examination revealed PPB [type II]