Prognostic factors in pleura-pulmonary blastoma

Paolo Indolfi, Gianni Bisogno, Fiorina Casale, Giovanni Cecchetto, Gianluca De Salvo, Andrea Ferrari, Alberto Donfrancesco, Vittoria Donofrio, Antonio Martone, Martina Di Martino, Maria T. Di Tullio

Research output: Contribution to journalArticle

Abstract

Purpose. To evaluate the prognostic factors in a series of children affected by pleuropulmonary blastoma (PPB). Patients and Methods. Clinicopathological findings, treatment, and outcome of 22 PPB cases observed in 13 Italian Associations for Pediatric Hematology and Oncology centers are reported. Clinical data, surgical notes, pathologic findings, and summaries of treatment were taken from the charts and correlated with outcome by standard statistical methods. Results. The series included 22 patients (14 males) with a median age of 30.5 months followed up for a median of 22 months (range 2-176 months). In nine patients the PPB developed with lung involvement only. Congenital lung cysts were recorded in five cases. Nine patients had recurrences. Gender, side, tumor size, pre-existing lung cysts, and extent of surgical resection at diagnosis did not significantly affect survival by univariate analysis. Achieving total resection of the tumor at any time of treatment resulted in a significantly better prognosis (P = 0.01), whereas extrapulmonary involvement at diagnosis resulted in a significantly worse prognosis (P = 0.01). Estimated 15-year event-free and overall survival rates were 44 and 49% for all patients, respectively. Conclusions. PPB is an aggressive neoplasm. Total resection of PPB performed at any time of treatment appears to provide a better outcome, whereas extrapulmonary involvement at diagnosis worsens the prognosis.

Original languageEnglish
Pages (from-to)318-323
Number of pages6
JournalPediatric Blood and Cancer
Volume48
Issue number3
DOIs
Publication statusPublished - Mar 2007

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Keywords

  • Children
  • Pleuro-pulmonary blastoma
  • Prognostic factors
  • Rare tumors

ASJC Scopus subject areas

  • Cancer Research
  • Pediatrics, Perinatology, and Child Health
  • Hematology

Cite this

Indolfi, P., Bisogno, G., Casale, F., Cecchetto, G., De Salvo, G., Ferrari, A., Donfrancesco, A., Donofrio, V., Martone, A., Di Martino, M., & Di Tullio, M. T. (2007). Prognostic factors in pleura-pulmonary blastoma. Pediatric Blood and Cancer, 48(3), 318-323. https://doi.org/10.1002/pbc.20842