title: Proton radiation therapy for pediatric medulloblastoma and supratentorial primitive neuroectodermal tumors: outcomes for very young children treated with upfront chemotherapy.
abstract: OBJECTIVE
  To report the early outcomes for very young children with medulloblastoma or supratentorial primitive neuroectodermal tumor (SPNET) treated with upfront chemotherapy followed by 3-dimensional proton radiation therapy (3D-CPT).
  
  
  METHODS
  All patients aged <60 months with medulloblastoma or SPNET treated with chemotherapy before 3D-CPT from 2002 to 2010 at our institution were included. All patients underwent maximal surgical resection, chemotherapy, and adjuvant 3D-CPT with either craniospinal irradiation followed by involved-field radiation therapy or involved-field radiation therapy alone.
  
  
  RESULTS
  Fifteen patients (median age at diagnosis, 35 months) were treated with high-dose chemotherapy and 3D-CPT. Twelve of 15 patients had medulloblastoma; 3 of 15 patients had SPNET. Median time from surgery to initiation of radiation was 219 days. Median craniospinal irradiation dose was 21.6 Gy (relative biologic effectiveness); median boost dose was 54.0 Gy (relative biologic effectiveness). At a median of 39 months from completion of radiation, 1 of 15 was deceased after a local failure, 1 of 15 had died from a non-disease-related cause, and the remaining 13 of 15 patients were alive without evidence of disease recurrence. Ototoxicity and endocrinopathies were the most common long-term toxicities, with 2 of 15 children requiring hearing aids and 3 of 15 requiring exogenous hormones.
  
  
  CONCLUSIONS
  Proton radiation after chemotherapy resulted in good disease outcomes for a small cohort of very young patients with medulloblastoma and SPNET. Longer follow-up and larger numbers of patients are needed to assess long-term outcomes and late toxicity.
authors.affiliation: Harvard Radiation Oncology Program, Boston, Massachusetts, USA. rbjimenez@partners.org
authors.forename: Rachel B
authors.initials: RB
authors.lastname: Jimenez
authors.forename: Roshan
authors.initials: R
authors.lastname: Sethi
authors.forename: Nicolas
authors.initials: N
authors.lastname: Depauw
authors.forename: Margaret B
authors.initials: MB
authors.lastname: Pulsifer
authors.forename: Judith
authors.initials: J
authors.lastname: Adams
authors.forename: Sean M
authors.initials: SM
authors.lastname: McBride
authors.forename: David
authors.initials: D
authors.lastname: Ebb
authors.forename: Barbara C
authors.initials: BC
authors.lastname: Fullerton
authors.forename: Nancy J
authors.initials: NJ
authors.lastname: Tarbell
authors.forename: Torunn I
authors.initials: TI
authors.lastname: Yock
authors.forename: Shannon M
authors.initials: SM
authors.lastname: Macdonald
mesh_terms: D001827:Body Height; D002423:Cause of Death; D002528:Cerebellar Neoplasms / Q000188:drug therapy / Q000401:mortality / Q000532:radiotherapy*; D017024:Chemotherapy, Adjuvant; D002675:Child, Preschool; D016371:Cranial Irradiation / Q000009:adverse effects / Q000379:methods; D004700:Endocrine System Diseases / Q000209:etiology; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000209:etiology; D006801:Humans; D007223:Infant; D008297:Male; D008527:Medulloblastoma / Q000188:drug therapy / Q000401:mortality / Q000532:radiotherapy*; D018242:Neuroectodermal Tumors, Primitive / Q000188:drug therapy / Q000401:mortality / Q000532:radiotherapy*; D061766:Proton Therapy / Q000009:adverse effects / Q000379:methods*; D011879:Radiotherapy Dosage; D015173:Supratentorial Neoplasms / Q000188:drug therapy / Q000401:mortality / Q000532:radiotherapy*; D016896:Treatment Outcome