Abstract

Pleuropulmonary blastoma (PPB) is a rare aggressive malignant tumor of infancy and early childhood accounting for 0.25-0.5% of primary lung tumors. PPB is a dysontogenic tumor composed of immature malignant epithelial and/or mesenchymal tissues whose features may resemble early embryological lung tissues. It is the pulmonary analog of other tumors of childhood including Wilms' tumor, neuroblastoma, hepatoblastoma, pancreatoblastoma and retinoblastoma. Morphologically, PPB has three types (I, II, and III). A fourth type (Ir) was added in 2006 by PPB registry. Type II and III are very aggressive malignancies with metastatic potential. Bilateral PPB is very rare. To the best of our knowledge, only two cases of bilateral PPB have been reported. No cases of bilateral PPB have been reported from India. Herein, we are reporting a case of bilateral PPB (type II) in a 2-year-old girl who presented with cough, dyspnea and mild fever for past 4 months. She died few days after admission.

Key words: Bilateral pleuropulmonary blastoma, Bronchogenic cyst, Congenital pulmonary airway malformations, Dysontogenic tumor, PPB registry, Primitive neuroectodermal tumors