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Diagnosis and treatment of pleuropulmonary blastoma—single center experience
Author(s) -
Christosova Iskra R.,
Avramova Boryana E.,
Drebov Rosen S.,
Shivachev Hristo I.,
Kamenova Margarita A.,
Bobev Dragan G.,
Brankov Ognyan G.
Publication year - 2015
Publication title -
pediatric pulmonology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.866
H-Index - 106
eISSN - 1099-0496
pISSN - 8755-6863
DOI - 10.1002/ppul.23047
Subject(s) - medicine , incidence (geometry) , disease , pediatrics , overall survival , single center , radiation therapy , surgery , physics , optics
Summary 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. Pediatr Pulmonol. 2015; 50:698–703. © 2014 Wiley Periodicals, Inc.