title: An individual patient data meta-analysis on characteristics and outcome of patients with papillary glioneuronal tumor, rosette glioneuronal tumor with neuropil-like islands and rosette forming glioneuronal tumor of the fourth ventricle.
abstract: OBJECTIVE
  In 2007, the WHO classification of brain tumors was extended by three new entities of glioneuronal tumors: papillary glioneuronal tumor (PGNT), rosette-forming glioneuronal tumor of the fourth ventricle (RGNT) and glioneuronal tumor with neuropil-like islands (GNTNI). Focusing on clinical characteristics and outcome, the authors performed a comprehensive individual patient data (IPD) meta-analysis of the cases reported in literature until December 2012.
  
  
  METHODS
  PubMed, Embase and Web of Science were searched for peer-reviewed articles reporting on PGNT, RGNT, and GNTNI using predefined keywords.
  
  
  RESULTS
  95 publications reported on 182 patients (PGNT, 71; GNTNI, 26; RGNT, 85). Median age at diagnosis was 23 years (range 4-75) for PGNT, 27 years (range 6-79) for RGNT, and 40 years (range 2-65) for GNTNI. Ninety-seven percent of PGNT and 69% of GNTNI were located in the supratentorial region, 23% of GNTNI were in the spinal cord, and 80% of RGNT were localized in the posterior fossa. Complete resection was reported in 52 PGNT (73%), 36 RGNT (42%), and 7 GNTNI (27%) patients. Eight PGNT, 3 RGNT, and 12 GNTNI patients were treated with chemo- and/or radiotherapy as the primary postoperative treatment. Follow-up data were available for 132 cases. After a median follow-up time of 1.5 years (range 0.2-25) across all patients, 1.5-year progression-free survival rates were 52±12% for GNTNI, 86±5% for PGNT, and 100% for RGNT. The 1.5-year overall-survival were 95±5%, 98±2%, and 100%, respectively.
  
  
  CONCLUSIONS
  The clinical understanding of the three new entities of glioneuronal tumors, PGNT, RGNT and GNTNI, is currently emerging. The present meta-analysis will hopefully contribute to a delineation of their diagnostic, therapeutic, and prognostic profiles. However, the available data do not provide a solid basis to define the optimum treatment approach. Hence, a central register should be established.
authors.affiliation: Department for Radiation Oncology, University of Leipzig Medical Center, Leipzig, Saxony, Germany.
authors.forename: Annika
authors.initials: A
authors.lastname: Schlamann
authors.affiliation: Department of Pediatrics and Adolescent Medicine, Division of Pediatric Hematology and Oncology, University of Göttingen Medical Center, Göttingen, Lower Saxony, Germany.
authors.forename: André O
authors.initials: AO
authors.lastname: von Bueren
authors.affiliation: Department of Neuropathology, University of Hamburg Eppendorf Medical Center, Hamburg, Germany.
authors.forename: Christian
authors.initials: C
authors.lastname: Hagel
authors.affiliation: Institute for Medical Biostatistics, Epidemiology and Informatics, University of Mainz Medical Center, Mainz, Rhineland-Palatinate, Germany.
authors.forename: Isabella
authors.initials: I
authors.lastname: Zwiener
authors.affiliation: Department for Radiation Oncology, University of Leipzig Medical Center, Leipzig, Saxony, Germany.
authors.forename: Clemens
authors.initials: C
authors.lastname: Seidel
authors.affiliation: Department for Radiation Oncology, University of Leipzig Medical Center, Leipzig, Saxony, Germany.
authors.forename: Rolf-Dieter
authors.initials: RD
authors.lastname: Kortmann
authors.affiliation: Department for Radiation Oncology, University of Leipzig Medical Center, Leipzig, Saxony, Germany.
authors.forename: Klaus
authors.initials: K
authors.lastname: Müller
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001932:Brain Neoplasms / Q000175:diagnosis* / Q000401:mortality / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D016208:Databases, Factual; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D005910:Glioma / Q000175:diagnosis* / Q000401:mortality / Q000473:pathology; D006801:Humans; D053208:Kaplan-Meier Estimate; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D018302:Neoplasms, Neuroepithelial / Q000175:diagnosis* / Q000401:mortality / Q000473:pathology; D011379:Prognosis; D016016:Proportional Hazards Models; D055815:Young Adult