TY - JOUR
T1 - Prognostic factors in pleura-pulmonary blastoma
AU - Indolfi, Paolo
AU - Bisogno, Gianni
AU - Casale, Fiorina
AU - Cecchetto, Giovanni
AU - De Salvo, Gianluca
AU - Ferrari, Andrea
AU - Donfrancesco, Alberto
AU - Donofrio, Vittoria
AU - Martone, Antonio
AU - Di Martino, Martina
AU - Di Tullio, Maria T.
PY - 2007/3
Y1 - 2007/3
N2 - 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.
AB - 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.
KW - Children
KW - Pleuro-pulmonary blastoma
KW - Prognostic factors
KW - Rare tumors
UR - http://www.scopus.com/inward/record.url?scp=33846932286&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=33846932286&partnerID=8YFLogxK
U2 - 10.1002/pbc.20842
DO - 10.1002/pbc.20842
M3 - Article
C2 - 16619221
AN - SCOPUS:33846932286
VL - 48
SP - 318
EP - 323
JO - Pediatric Blood and Cancer
JF - Pediatric Blood and Cancer
SN - 1545-5009
IS - 3
ER -