title: Whole neuraxis irradiation to address central nervous system relapse in high-risk neuroblastoma.
abstract: BACKGROUND
  As systemic control of high-risk neuroblastoma (NB) has improved, relapse in the central nervous system (CNS) is an increasingly recognized entity that carries a grim prognosis. This study describes the use of craniospinal irradiation (CSI) for CNS relapse and compares outcomes to patients who received focal radiotherapy (RT).
  
  
  METHODS
  A retrospective query identified 29 children with NB treated at Memorial Sloan-Kettering Cancer Center since 1987 who received RT for CNS relapse. At CNS relapse, 16 patients received CSI (median dose, 2160cGy), and 13 received focal RT. Of those who underwent CSI, 14 (88%) received intra-Ommaya (IO) radioimmunotherapy (RIT); one patient in the non-CSI cohort received IO-RIT.
  
  
  RESULTS
  Patient characteristics were similar between the groups. Time to CNS relapse was 20 and 17 months for the CSI and non-CSI cohorts, respectively. At a median follow-up of 28 months, 12 patients (75%) in the CSI group are alive without CNS disease, including two patients with isolated skeletal relapse. Another patient is alive without disease after a brain relapse was retreated with RT. Three patients died-one with no NB at autopsy, one of CNS disease, and one of systemic disease. The two patients who died of NB did not receive IO-RIT. All 13 patients in the non-CSI cohort died at a median of 8.8 months.
  
  
  CONCLUSIONS
  Low-dose CSI together with IO-RIT provides durable CNS remissions and improved survival compared with focal RT and conventional therapies. Further evaluation of long-term NB survivors after CSI is warranted to determine the treatment consequences for this cohort.
authors.affiliation: Department of Radiation Oncology, Memorial Sloan-Kettering Cancer Center, New York, NY, USA. vcroog@sibley.org
authors.forename: Victoria J
authors.initials: VJ
authors.lastname: Croog
authors.forename: Kim
authors.initials: K
authors.lastname: Kramer
authors.forename: Nai-Kong V
authors.initials: NK
authors.lastname: Cheung
authors.forename: Brian H
authors.initials: BH
authors.lastname: Kushner
authors.forename: Shakeel
authors.initials: S
authors.lastname: Modak
authors.forename: Mark M
authors.initials: MM
authors.lastname: Souweidane
authors.forename: Suzanne L
authors.initials: SL
authors.lastname: Wolden
mesh_terms: D000293:Adolescent; D001932:Brain Neoplasms / Q000401:mortality / Q000532:radiotherapy* / Q000556:secondary; D002648:Child; D002675:Child, Preschool; D016371:Cranial Irradiation / Q000379:methods* / Q000401:mortality; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D008577:Meningeal Neoplasms / Q000401:mortality / Q000532:radiotherapy* / Q000556:secondary; D009447:Neuroblastoma / Q000401:mortality / Q000532:radiotherapy* / Q000556:secondary; D016499:Radioimmunotherapy / Q000379:methods* / Q000401:mortality; D012074:Remission Induction / Q000379:methods; D012189:Retrospective Studies; D013120:Spinal Cord Neoplasms / Q000401:mortality / Q000532:radiotherapy* / Q000556:secondary; D013997:Time Factors; D016896:Treatment Outcome