It's Rare So Be Aware

Pleuropulmonary Blastoma Mimicking Congenital Pulmonary Airway Malformation:

THORAC CARDIOV SURG Reports 2017; 06: e10-e14
DOI: 10.1055/s-0037-1598625

Pleuropulmonary blastoma (PPB) 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. Four types are defined in literature. Type I PPB is a rare, cystic lung neoplasm in infants characterized by subtle malignant changes and a good prognosis. Recurrences after type I PPB are usually advanced with a poor prognosis.We report this case to increase awareness about this entity so that the pediatricians, pediatric surgeons, radiologist, and pathologist recognize it early.
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Georg Thieme Verlag KG Stuttgart · New York

Article in Thieme eJournals:
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