title: Brain stem tumors in children and adolescents: single institutional experience.
abstract: OBJECTIVE
  Pediatric brain stem tumors (BsT) are a heterogeneous group of diseases. Our aim was to analyze our experience to find out prognostic factors.
  
  
  METHODS
  A retrospective study with BsT patients was performed. Imaging characteristics, extension of surgery, pathology, and adjuvant therapy were analyzed and correlated with overall survival (OS) and progression-free survival (PFS) as outcome measures.
  
  
  RESULTS
  Since 1980 to 2010, we analyzed 65 BsT patients, 41 of them girls (63%), median age of 8 years (range 13.9 months to 17.6 years). Twenty-two patients (33.8%) had diffuse intrinsic pontine gliomas (DIPG) and 43 (66.2%) presented with focal BsT. Histology was available in 42 patients; the most frequent is low-grade glioma in 24/42 patients (57%). DIPG's histology (obtained usually at necropsy) confirmed five high-grade gliomas. After median follow-up of 49.3 months (0.5-175 months), 20/22 DIPG patients have died (90.9%), while 27/43 with focal tumors were alive (62.8%). Variables related to outcome were histology (better for low-grade glioma (LGG) OS p < 0.001), surgery (better if operated OS p < 0.001), and adjuvant therapy (worse if given, PFS p = 0.001, OS p = 0.024). The outcome for DIPG was dismal, median OS/EFS of 14.2/9.4 months, significantly worse than focal BsT (p = 0.000), while OS/EFS was 122.8/87.2 months for focal intrinsic, 88.2/47.1 months for exophytic, and 124.4/54 months for cervico-medullary tumors: no differences were found among them, except the histology (OS p < 0.001 for low-grade vs high-grade tumors).
  
  
  CONCLUSIONS
  BsT in children comprised two different groups: diffuse (DIPG) and focal gliomas. The DIPGs continue having a dismal prognosis, needing new approaches, while focal tumors including LGG have better prognosis.
authors.affiliation: Department of Pediatric Neurosurgery, Hospital Sant Joan de Déu, University of Barcelona, Passeig St. Joan de Déu, 2, Esplugues de Llobregat, 08950 Barcelona, Spain. dendrita07@gmail.com
authors.forename: Magda
authors.initials: M
authors.lastname: Garzón
authors.forename: Gemma
authors.initials: G
authors.lastname: García-Fructuoso
authors.forename: Antonio
authors.initials: A
authors.lastname: Guillén
authors.forename: Mariona
authors.initials: M
authors.lastname: Suñol
authors.forename: Jaume
authors.initials: J
authors.lastname: Mora
authors.forename: Ofelia
authors.initials: O
authors.lastname: Cruz
mesh_terms: D000293:Adolescent; D017668:Age of Onset; D020295:Brain Stem Neoplasms / Q000145:classification / Q000150:complications / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy*; D002648:Child; D002675:Child, Preschool; D003072:Cognition Disorders / Q000209:etiology; D003131:Combined Modality Therapy; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006849:Hydrocephalus / Q000209:etiology; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D017063:Outcome Assessment, Health Care; D011183:Postoperative Complications; D012189:Retrospective Studies