Pleuropulmonary blastoma in a 12-year-old boy presenting with pneumothorax

Numanami, Hiroki; Tanaka, Motoya; Hashizume, Masahiro; Hara, Kazuo; Yokoi, Toyoharu; Haniuda, Masayuki

doi:10.1007/s11748-006-0044-z

Cited by 6 publications

(1 citation statement)

References 8 publications

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“…Two (18%) of our patients were diagnosed at more than 6‐year of age (both patients were 12 years old). This is very uncommon, but similar cases have been described in the literature . According to clinical characteristics, most of our patients had type II and III disease (91%), with right pulmonary localization (63.6%), in the middle and inferior lobes (63.6%).…”

Section: Discussionsupporting

confidence: 79%

Diagnosis and treatment of pleuropulmonary blastoma—single center experience

Christosova¹,

Avramova²,

Drebov

et al. 2014

Pediatric Pulmonology

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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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Section: Discussionsupporting

confidence: 79%