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Pleuropulmonary blastoma, a distinctive neoplasm of childhood: report of three cases.

Orazi C, Inserra A, Schingo PM, De Sio L, Cutrera R, Boldrini R, Malena S

Department of Diagnostic Imaging, I.R.C.C.S. Bambino Gesù Pediatric Hospital, P.za S. Onofrio 4, 00165, Rome, Italy.

BACKGROUND: Pleuropulmonary blastoma (PPB) is a rare, aggressive dysontogenetic neoplasm affecting children. It was identified as a distinct entity by Manivel in 1988 and later subdivided into three types on the basis of the histological pattern, with increasing malignancy from type I (cystic) through type II (solid/cystic) to type III (solid). OBJECTIVE: To report on the imaging findings, clinical presentation, and differential diagnosis, mainly cystic malformations. MATERIALS AND METHODS: We evaluated three children, age 2-4 years, with PPB. RESULTS: One patient presented with unresolving pneumothorax and a multicystic mass, another with a mixed fluid/solid lesion, and the last with a solid heterogeneous mass. CONCLUSION: Despite its rarity, PPB should be considered in the evaluation of cystic or solid masses in children with respiratory distress. Plain film radiography alone is unable to distinguish between PPB and cystic malformations. CT represents the gold standard, although MRI can show the imaging features of solid enhancing nodules inside fluid-filled cavities, a mass causing lung compression, mediastinal shift, frequent pleural effusion, and no chest wall invasion. No preoperative imaging can reliably differentiate between congenital cystic lesions and PPB type I.

Published 6 March 2007 in Pediatr Radiol, 37(4): 337-44.
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