Diagnosis and treatment of pleuropulmonary blastoma-single center experience.
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| Abstract |
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.
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| Authors | Iskra R Christosova, Boryana E Avramova, Rosen S Drebov, Hristo I Shivachev, Margarita A Kamenova, Dragan G Bobev, Ognyan G Brankov |
| Journal | Pediatric pulmonology (Pediatr Pulmonol) Vol. 50 Issue 7 Pg. 698-703 (Jul 2015) ISSN: 1099-0496 [Electronic] United States |
| PMID | 24692196 (Publication Type: Journal Article) |
| Copyright | © 2014 Wiley Periodicals, Inc. |
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