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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.

Author information

1
Specialized Hospital for Pediatric Oncohematology, Sofia, Sofia, Bulgaria.
2
University Hospital for Emergency Medicine "Pirogov," Sofia, Clinic of Pediatric Surgery, Sofia, Bulgaria.
3
Section of Pathology, University Hospital for Emergency Medicine "Pirogov," Sofia, Sofia, Bulgaria.

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.

KEYWORDS:

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

PMID:
24692196
DOI:
10.1002/ppul.23047
[Indexed for MEDLINE]
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