title: Diagnosis and treatment of pleuropulmonary blastoma-single center experience.
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.
authors.affiliation: Specialized Hospital for Pediatric Oncohematology, Sofia, Sofia, Bulgaria.
authors.forename: Iskra R
authors.initials: IR
authors.lastname: Christosova
authors.affiliation: Specialized Hospital for Pediatric Oncohematology, Sofia, Sofia, Bulgaria.
authors.forename: Boryana E
authors.initials: BE
authors.lastname: Avramova
authors.affiliation: University Hospital for Emergency Medicine "Pirogov," Sofia, Clinic of Pediatric Surgery, Sofia, Bulgaria.
authors.forename: Rosen S
authors.initials: RS
authors.lastname: Drebov
authors.affiliation: University Hospital for Emergency Medicine "Pirogov," Sofia, Clinic of Pediatric Surgery, Sofia, Bulgaria.
authors.forename: Hristo I
authors.initials: HI
authors.lastname: Shivachev
authors.affiliation: Section of Pathology, University Hospital for Emergency Medicine "Pirogov," Sofia, Sofia, Bulgaria.
authors.forename: Margarita A
authors.initials: MA
authors.lastname: Kamenova
authors.affiliation: Specialized Hospital for Pediatric Oncohematology, Sofia, Sofia, Bulgaria.
authors.forename: Dragan G
authors.initials: DG
authors.lastname: Bobev
authors.affiliation: University Hospital for Emergency Medicine "Pirogov," Sofia, Clinic of Pediatric Surgery, Sofia, Bulgaria.
authors.forename: Ognyan G
authors.initials: OG
authors.lastname: Brankov
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use; D002031:Bulgaria / Q000453:epidemiology; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008175:Lung Neoplasms / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy*; D008297:Male; D011013:Pneumonectomy; D011379:Prognosis; D018202:Pulmonary Blastoma / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy*; D012189:Retrospective Studies
chemical_list: D000970:Antineoplastic Agents
keywords: combined modality treatment; dysembryonic neoplasm; myeloablative chemotherapy; pleuropulmonary blastoma