title: Diffuse intrinsic pontine glioma in children and adolescents: a single-center experience.
abstract: BACKGROUND
  Patients with diffuse intrinsic pontine glioma (DIPG) have a very poor prognosis. Only radiotherapy (XRT) has proven to be effective in delaying the disease progression. Several chemotherapy schedules have been applied so far, but none demonstrated significant improvements in progression and survival.
  
  
  METHODS
  We retrospectively analyzed the clinical data of children diagnosed with DIPG at our center (Pediatric Hospital "Regina Margherita," Turin, Italy) between 1999 and 2013. Progression-free survival (PFS) and overall survival (OS) were used to describe the outcomes.
  
  
  RESULTS
  Twenty-four children were included in our report. Patients diagnosed before March 2003 (n = 12) were treated with XRT and vincristine (VCR); the remaining 12 patients received XRT and temozolomide (TMZ). Progression-free survival was 18.8 % at 1 year (SE = 7.6 %), while overall survival was 44.1 % at 1 year (SE = 9.9 %). Median PFS was 8.1 months, whereas median OS was 11.2 months. No statistically significant difference in PFS or OS was evidenced between the two treatment groups.
  
  
  CONCLUSIONS
  Radiotherapy followed by VCR or TMZ allows obtaining results that are in line with previous reports, with no advantages over other similar treatment schedules. DIPGs are challenging tumors with a dismal outcome. Further research and newer therapies are urgently needed in order to achieve improvements in survival.
authors.affiliation: Pediatric Oncohematology, Stem Cell Transplantation and Cell Therapy Division, A.O. Città della Salute e della Scienza-Ospedale Infantile "Regina Margherita", Piazza Polonia, 94, 10126, Turin, Italy, stefano.vallero@gmail.com.
authors.forename: Stefano Gabriele
authors.initials: SG
authors.lastname: Vallero
authors.forename: Daniele
authors.initials: D
authors.lastname: Bertin
authors.forename: Maria Eleonora
authors.initials: ME
authors.lastname: Basso
authors.forename: Laura Stefania
authors.initials: LS
authors.lastname: Pittana
authors.forename: Anna
authors.initials: A
authors.lastname: Mussano
authors.forename: Franca
authors.initials: F
authors.lastname: Fagioli
mesh_terms: D000293:Adolescent; D018906:Antineoplastic Agents, Alkylating; D020295:Brain Stem Neoplasms / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy*; D002648:Child; D003606:Dacarbazine / Q000031:analogs & derivatives / Q000627:therapeutic use; D005260:Female; D005910:Glioma / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy*; D006801:Humans; D008297:Male; D011149:Pons / Q000473:pathology*; D012189:Retrospective Studies; D016019:Survival Analysis; D000077204:Temozolomide
chemical_list: D018906:Antineoplastic Agents, Alkylating; D003606:Dacarbazine; D000077204:Temozolomide