Pleuropulmonary Blastoma: Case Report

Abstract

Pleuropulmonary blastoma (PPB) is a rare and aggressive tumor that is emerging as a distinct entity of early childhood disease. It is characterized by mesenchymal elements (including undifferentiated blastoma and often cartilaginous, rhabdomyoblastic, or fibroblastic differentiation) and epithelium-lined spaces. The tumor arises in the lung and pleura and is regarded as a pulmonary dysontogenetic or embryonic neoplasm. It is the
pulmonary analog of other tumors of childhood including Wilms` tumor, Neuroblastoma, Hepatoblastoma, Pancreatoblastoma and Retinoblastoma. Due to their protean presentation it is often difficult to make a preoperative diagnosis. A high index of suspicion therefore is needed. As a result these are diagnosed late, and these, along with other factors, affect the eventual outcome. We report a case of Pleuropulmonary blastoma diagnosed after the child was operated as a case of massive left hemothorax following blunt
trauma.