Prognostic factors in pleuro-pulmonary blastoma.
OBJECTIVE
To evaluate the prognostic factors in a series of children affected by pleuropulmonary blastoma (PPB).


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.
Indolfi
Paolo
P
Pediatric Oncology Service Second University of Naples, Italy. paolo.indolfi@unina2.it
Bisogno
Gianni
G
Casale
Fiorina
F
Cecchetto
Giovanni
G
De Salvo
Gianluca
G
Ferrari
Andrea
A
Donfrancesco
Alberto
A
Donofrio
Vittoria
V
Martone
Antonio
A
Di Martino
Martina
M
Di Tullio
Maria T
MT
D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage / Q000627:therapeutic use; D016190:Carboplatin / Q000008:administration & dosage; D017024:Chemotherapy, Adjuvant; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D003609:Dactinomycin / Q000008:administration & dosage; D018450:Disease Progression; D018572:Disease-Free Survival; D004317:Doxorubicin / Q000008:administration & dosage; D015251:Epirubicin / Q000008:administration & dosage; D005047:Etoposide / Q000008:administration & dosage; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007069:Ifosfamide / Q000008:administration & dosage; D007223:Infant; D053208:Kaplan-Meier Estimate; D008175:Lung Neoplasms / Q000188:drug therapy / Q000401:mortality* / Q000473:pathology / Q000532:radiotherapy / Q000601:surgery; D008297:Male; D009362:Neoplasm Metastasis; D009367:Neoplasm Staging; D010997:Pleural Neoplasms / Q000188:drug therapy / Q000401:mortality* / Q000473:pathology / Q000532:radiotherapy / Q000601:surgery; D011013:Pneumonectomy; D011379:Prognosis; D018202:Pulmonary Blastoma / Q000188:drug therapy / Q000401:mortality* / Q000473:pathology / Q000532:radiotherapy / Q000601:surgery; D018714:Radiotherapy, Adjuvant; D012189:Retrospective Studies; D012307:Risk Factors; D024162:Second-Look Surgery; D016896:Treatment Outcome; D014750:Vincristine / Q000008:administration & dosage
D003609:Dactinomycin; D015251:Epirubicin; D014750:Vincristine; D005047:Etoposide; D004317:Doxorubicin; D016190:Carboplatin; D007069:Ifosfamide