Abstract

Note Pleuropulmonary blastoma is a rare aggressive malignant tumor of infancy and early childhood. 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. Synonyms include embryonal rhabomyosarcoma arising in congenital bronchogenic cyst, rhabdomyosarcoma arising in congenital cystic adenomatoid malformation, pulmonary sarcoma arising in mesenchymal cystic hamartoma, pulmonary blastoma associated with cystic lung disease of childhood.

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