Prognostic factors in pleuro-pulmonary blastoma
Article first published online: 17 APR 2006
Copyright © 2006 Wiley-Liss, Inc.
Pediatric Blood & Cancer
Volume 48, Issue 3, pages 318–323, March 2007
How to Cite
Indolfi, P., Bisogno, G., Casale, F., Cecchetto, G., De Salvo, G., Ferrari, A., Donfrancesco, A., Donofrio, V., Martone, A., Di Martino, M. and Di Tullio, M. T. (2007), Prognostic factors in pleuro-pulmonary blastoma. Pediatr. Blood Cancer, 48: 318–323. doi: 10.1002/pbc.20842
- Issue published online: 3 JAN 2007
- Article first published online: 17 APR 2006
- Manuscript Accepted: 24 FEB 2006
- Manuscript Received: 14 SEP 2005
- pleuro-pulmonary blastoma;
- prognostic factors;
- rare tumors
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.
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.
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. Pediatr Blood Cancer 2007;48:318–323. © 2006 Wiley-Liss, Inc.