title: Hypofractionated stereotactic radiotherapy in the management of recurrent or residual medulloblastoma/PNET.
abstract: OBJECTIVE
  To evaluate the efficacy and toxicity of hypofractionated stereotactic radiotherapy in the management of locally recurrent or residual central nervous system (CNS) primitive neuroectodermal tumors (PNETs).
  
  
  METHODS
  Between 1991 and 2005, 12 patients with locally recurrent medulloblastoma and two patients with residual supratentorial PNET were treated with hypofractionated stereotactic conformal radiotherapy (SCRT). Nine patients were treated for first recurrence, two patients after the 2nd, and one patient after 3rd recurrence. Median age at diagnosis was 20 years (range: 4-35 years) and median age at SCRT 25 years (range: 7-41 years). Nine of 12 patients underwent resection at recurrence and 13 patients received at least one cycle of chemotherapy prior to SCRT. All received focal SCRT (30-40 Gy/6-8 #) using non-coplanar arcs (n = 6) or fixed conformal non-coplanar fields (n = 8).
  
  
  RESULTS
  Median overall survival was 29 months (95% CI: 6-51 months) and median progression-free survival was 12 months (95% CI: 5-19 months). Local progression-free survival at 1 and 3 years was 80% (95% CI: 55-100%) and 48% (95% CI: 11-85%). Causes of death were recurrent CNS disease (n = 7), herpes encephalitis (n = 1), and metastatic PNET outside the CNS (n = 1).
  
  
  CONCLUSIONS
  Hypofractionated SCRT provides effective local control with acceptable toxicity for patients with recurrent localized PNET. However, overall long-term disease control is rare and limited by the occurrence of CSF mediated relapses, which thus could benefit from intensive systemic chemotherapy as part of the primary relapse strategy even in local recurrences. Larger multi-national studies will be necessary to assess the value of such combined treatment approaches.
authors.affiliation: Neuro-oncology Unit and the Academic Unit of Radiotherapy and Oncology, The Institute of Cancer Research and The Royal Marsden NHS Foundation Trust, Sutton, United Kingdom. Frank.Saran@rmh.nhs.uk
authors.forename: Frank
authors.initials: F
authors.lastname: Saran
authors.forename: Brigitta G
authors.initials: BG
authors.lastname: Baumert
authors.forename: Antonia L
authors.initials: AL
authors.lastname: Creak
authors.forename: Alan P
authors.initials: AP
authors.lastname: Warrington
authors.forename: Sue
authors.initials: S
authors.lastname: Ashley
authors.forename: Daphne
authors.initials: D
authors.lastname: Traish
authors.forename: Michael
authors.initials: M
authors.lastname: Brada
mesh_terms: D000293:Adolescent; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002528:Cerebellar Neoplasms / Q000188:drug therapy / Q000473:pathology / Q000532:radiotherapy* / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D019583:Dose Fractionation, Radiation; D005260:Female; D006801:Humans; D008297:Male; D008527:Medulloblastoma / Q000188:drug therapy / Q000473:pathology / Q000532:radiotherapy* / Q000601:surgery; D009364:Neoplasm Recurrence, Local / Q000532:radiotherapy; D018365:Neoplasm, Residual; D018242:Neuroectodermal Tumors, Primitive / Q000188:drug therapy / Q000473:pathology / Q000532:radiotherapy* / Q000601:surgery; D010166:Palliative Care; D020266:Radiotherapy, Conformal / Q000379:methods*; D012189:Retrospective Studies; D013238:Stereotaxic Techniques; D015173:Supratentorial Neoplasms / Q000188:drug therapy / Q000473:pathology / Q000532:radiotherapy* / Q000601:surgery