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Pediatr Pulmonol. 2015 Jul;50(7):698-703. doi: 10.1002/ppul.23047. Epub 2014 Apr 1.

Diagnosis and treatment of pleuropulmonary blastoma-single center experience.

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Specialized Hospital for Pediatric Oncohematology, Sofia, Sofia, Bulgaria.
University Hospital for Emergency Medicine "Pirogov," Sofia, Clinic of Pediatric Surgery, Sofia, Bulgaria.
Section of Pathology, University Hospital for Emergency Medicine "Pirogov," Sofia, Sofia, Bulgaria.


Pleuropulmonary blastoma (PPB) is a rare and potentially aggressive intrathoracic disembryonic neoplasm typically occurring in children less than 6 years of age. We assessed the relative incidence, clinical characteristics, treatment outcome, and the prognostic factors for long-term survival in patients with PPB treated at our institution over a 25-year period, and compared these data with reports in the literature. From 1985 to 2010, 11 children (4 males and 7 females), with a median age of 5.4 years (range, 1-12 years) were treated at our hospital. Here we described the main characteristics of these patients, the diagnostic methods, and treatment modalities used. During a median follow-up period of 80, 9 months, the overall survival (OS) and disease-free survival (DFS) rates were 54, 6% and 45, 5%, respectively. Two patients survived for more than 20 years. The main prognostic factors for long-term survival were the diseases type I and II and treatment with radical surgery. Our results show that in order to improve the prognosis of patients with PPB a timely in our opinion and accurate diagnosis needs to be established and treatment should be offered according to the disease type and extend of dissemination.


combined modality treatment; dysembryonic neoplasm; myeloablative chemotherapy; pleuropulmonary blastoma

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