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Children, Volume 6, Issue 8 (August 2019)
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Cover Story (view full-size image) Pleuropulmonary blastomas (PPB) are sarcomatous malignant pediatric tumors of the lung parenchyma [...] Read more. Pleuropulmonary blastomas (PPB) are sarcomatous malignant pediatric tumors of the lung parenchyma and pleural surfaces which fall under the larger aegis of the DICER1-related disorders. A PPB arises initially as a pulmonary cyst. Histologically, these cysts are lined with epithelium and also contain underlying sarcomatous mesenchyme. PPB undergo a well-documented progression through several forms which allows their division into types I, Ir, II, and III, with rough correlates to age at detection and prognosis. A consensus has not been reached regarding the optimal surgical management of PPB. Therefore, the authors aim to provide a comprehensive review of the current literature describing the characteristics of PPB and multidisciplinary management. View this paper.