title: Prognostic factors in pleuro-pulmonary blastoma.
abstract: 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.
authors.affiliation: Pediatric Oncology Service Second University of Naples, Italy. paolo.indolfi@unina2.it
authors.forename: Paolo
authors.initials: P
authors.lastname: Indolfi
authors.forename: Gianni
authors.initials: G
authors.lastname: Bisogno
authors.forename: Fiorina
authors.initials: F
authors.lastname: Casale
authors.forename: Giovanni
authors.initials: G
authors.lastname: Cecchetto
authors.forename: Gianluca
authors.initials: G
authors.lastname: De Salvo
authors.forename: Andrea
authors.initials: A
authors.lastname: Ferrari
authors.forename: Alberto
authors.initials: A
authors.lastname: Donfrancesco
authors.forename: Vittoria
authors.initials: V
authors.lastname: Donofrio
authors.forename: Antonio
authors.initials: A
authors.lastname: Martone
authors.forename: Martina
authors.initials: M
authors.lastname: Di Martino
authors.forename: Maria T
authors.initials: MT
authors.lastname: Di Tullio
mesh_terms: 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
chemical_list: D003609:Dactinomycin; D015251:Epirubicin; D014750:Vincristine; D005047:Etoposide; D004317:Doxorubicin; D016190:Carboplatin; D007069:Ifosfamide