title: Clinical significance of intensive surgery with intraoperative radiation for advanced neuroblastoma: does it really make sense?
abstract: OBJECTIVE
  The aim of this study was to evaluate the significance of intensive surgery combined with intraoperative radiation therapy (IORT) in advanced neuroblastoma.
  
  
  METHODS
  Clinical features and outcome were reviewed in 33 advanced neuroblastoma patients (24 with INSS stage 4, 9 with stage 3), who had surgery (total excision 29, subtotal excision 4) with IORT (10 to 15 Gy) against the primary tumor site.
  
  
  RESULTS
  Three patients (8.8%) had relapse at the primary site, all of which arose from the unirradiated area after stem cell transplantation. Among 29 patients with total excision, disease-free survival was obtained in 15 (51.7%) for an average of 6.9 years, which included 5 survivors of 9 patients (55.9%) with amplified N-myc. In contrast, none of 4 patients with macroscopic residual survived. The Kaplan-Meier analysis showed significantly longer survival rates in the patients with total resection compared with those with macroscopic remnants.
  
  
  CONCLUSIONS
  The intensive surgery with IORT dramatically increased the local eradication and improved the outcome even in advanced neuroblastoma with N-myc amplification. However, long-term survival was not obtained in patients with unresectable residual disease. These results may indicate the key role of surgical eradication in advanced neuroblastoma.
authors.affiliation: Department of Surgery, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: Tatsuo
authors.initials: T
authors.lastname: Kuroda
authors.forename: Morihiro
authors.initials: M
authors.lastname: Saeki
authors.forename: Toshiro
authors.initials: T
authors.lastname: Honna
authors.forename: Hidekazu
authors.initials: H
authors.lastname: Masaki
authors.forename: Yukiko
authors.initials: Y
authors.lastname: Tsunematsu
mesh_terms: D000310:Adrenal Gland Neoplasms / Q000532:radiotherapy* / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D018572:Disease-Free Survival; D005260:Female; D005784:Gene Amplification; D016259:Genes, myc; D006801:Humans; D007223:Infant; D008197:Lymph Node Excision; D008297:Male; D009364:Neoplasm Recurrence, Local; D009367:Neoplasm Staging; D018365:Neoplasm, Residual; D009447:Neuroblastoma / Q000235:genetics / Q000532:radiotherapy* / Q000601:surgery*; D012186:Retroperitoneal Neoplasms / Q000532:radiotherapy* / Q000601:surgery*; D012189:Retrospective Studies; D033581:Stem Cell Transplantation