title: Treatment of pulmonary metastases in children with stage IV nephroblastoma with risk-based use of pulmonary radiotherapy.
abstract: OBJECTIVE
  The purpose of this study was to determine the outcome of children with nephroblastoma and pulmonary metastases (PM) treated according to International Society of Pediatric Oncology (SIOP) 93-01 recommendations using pulmonary radiotherapy (RT) in selected patients.
  
  
  METHODS
  Patients (6 months to 18 years) were treated with preoperative chemotherapy consisting of 6 weeks of vincristine, dactinomycin, and epirubicin or doxorubicin. If pulmonary complete remission (CR) was not obtained, metastasectomy was considered. Patients in CR received three-drug postoperative chemotherapy, whereas patients not in CR were switched to a high-risk (HR) regimen with an assessment at week 11. If CR was not obtained, pulmonary RT was mandatory.
  
  
  RESULTS
  Two hundred thirty-four of 1,770 patients had PM. Patients with PM were older (P < .001) and had larger tumor volumes compared with nonmetastatic patients (P < .001). Eighty-four percent of patients were in CR postoperatively, with 17% requiring metastasectomy. Thirty-five patients (16%) had multiple inoperable PM and required the HR protocol. Only 14% of patients received pulmonary RT during first-line treatment. For patients with PM, 5-year event-free survival rate was 73% (95% CI, 68% to 79%), and 5-year overall survival (OS) rate was 82% (95% CI, 77% to 88%). Five-year OS was similar for patients with local stage I and II disease (92% and 90%, respectively) but lower for patients with local stage III disease (68%; P < .001). Patients in CR after chemotherapy only and patients in CR after chemotherapy and metastasectomy had a better outcome than patients with multiple unresectable PM (5-year OS, 88%, 92%, and 48%, respectively; P < .001).
  
  
  CONCLUSIONS
  Following the SIOP protocol, pulmonary RT can be omitted for a majority of patients with PM and results in a relatively good outcome.
authors.affiliation: Department of Pediatric Oncology, Hôpital d'Enfants de La Timone, 264, rue Saint Pierre, 13385 Marseille Cedex 5, France. arnauld.verschuur@mail.ap-hm.fr
authors.forename: Arnauld
authors.initials: A
authors.lastname: Verschuur
authors.forename: Harm
authors.initials: H
authors.lastname: Van Tinteren
authors.forename: Norbert
authors.initials: N
authors.lastname: Graf
authors.forename: Christophe
authors.initials: C
authors.lastname: Bergeron
authors.forename: Bengt
authors.initials: B
authors.lastname: Sandstedt
authors.forename: Jan
authors.initials: J
authors.lastname: de Kraker
mesh_terms: D000293:Adolescent; D017024:Chemotherapy, Adjuvant; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D006801:Humans; D007223:Infant; D007680:Kidney Neoplasms / Q000401:mortality / Q000473:pathology*; D008175:Lung Neoplasms / Q000188:drug therapy / Q000532:radiotherapy* / Q000556:secondary* / Q000601:surgery; D015996:Survival Rate; D009396:Wilms Tumor / Q000401:mortality / Q000473:pathology*