title: Description of selected characteristics of familial glioma patients - results from the Gliogene Consortium.
abstract: BACKGROUND
  While certain inherited syndromes (e.g. Neurofibromatosis or Li-Fraumeni) are associated with an increased risk of glioma, most familial gliomas are non-syndromic. This study describes the demographic and clinical characteristics of the largest series of non-syndromic glioma families ascertained from 14 centres in the United States (US), Europe and Israel as part of the Gliogene Consortium.
  
  
  METHODS
  Families with 2 or more verified gliomas were recruited between January 2007 and February 2011. Distributions of demographic characteristics and clinical variables of gliomas in the families were described based on information derived from personal questionnaires.
  
  
  RESULTS
  The study population comprised 841 glioma patients identified in 376 families (9797 individuals). There were more cases of glioma among males, with a male to female ratio of 1.25. In most families (83%), 2 gliomas were reported, with 3 and 4 gliomas in 13% and 3% of the families, respectively. For families with 2 gliomas, 57% were among 1st-degree relatives, and 31.5% among 2nd-degree relatives. Overall, the mean (±standard deviation [SD]) diagnosis age was 49.4 (±18.7) years. In 48% of families with 2 gliomas, at least one was diagnosed at <40y, and in 12% both were diagnosed under 40y of age. Most of these families (76%) had at least one grade IV glioblastoma multiforme (GBM), and in 32% both cases were grade IV gliomas. The most common glioma subtype was GBM (55%), followed by anaplastic astrocytoma (10%) and oligodendroglioma (8%). Individuals with grades I-II were on average 17y younger than those with grades III-IV.
  
  
  CONCLUSIONS
  Familial glioma cases are similar to sporadic cases in terms of gender distribution, age, morphology and grade. Most familial gliomas appear to comprise clusters of two cases suggesting low penetrance, and that the risk of developing additional gliomas is probably low. These results should be useful in the counselling and clinical management of individuals with a family history of glioma.
authors.affiliation: Cancer and Radiation Epidemiology Unit, Gertner Institute, Chaim Sheba Medical Center, Tel Hashomer, Israel. siegals@gertner.health.gov.il
authors.forename: Siegal
authors.initials: S
authors.lastname: Sadetzki
authors.forename: Revital
authors.initials: R
authors.lastname: Bruchim
authors.forename: Bernice
authors.initials: B
authors.lastname: Oberman
authors.forename: Georgina N
authors.initials: GN
authors.lastname: Armstrong
authors.forename: Ching C
authors.initials: CC
authors.lastname: Lau
authors.forename: Elizabeth B
authors.initials: EB
authors.lastname: Claus
authors.forename: Jill S
authors.initials: JS
authors.lastname: Barnholtz-Sloan
authors.forename: Dora
authors.initials: D
authors.lastname: Il'yasova
authors.forename: Joellen
authors.initials: J
authors.lastname: Schildkraut
authors.forename: Christoffer
authors.initials: C
authors.lastname: Johansen
authors.forename: Richard S
authors.initials: RS
authors.lastname: Houlston
authors.forename: Sanjay
authors.initials: S
authors.lastname: Shete
authors.forename: Christopher I
authors.initials: CI
authors.lastname: Amos
authors.forename: Jonine L
authors.initials: JL
authors.lastname: Bernstein
authors.forename: Sara H
authors.initials: SH
authors.lastname: Olson
authors.forename: Robert B
authors.initials: RB
authors.lastname: Jenkins
authors.forename: Daniel
authors.initials: D
authors.lastname: Lachance
authors.forename: Nicholas A
authors.initials: NA
authors.lastname: Vick
authors.forename: Ryan
authors.initials: R
authors.lastname: Merrell
authors.forename: Margaret
authors.initials: M
authors.lastname: Wrensch
authors.forename: Faith G
authors.initials: FG
authors.lastname: Davis
authors.forename: Bridget J
authors.initials: BJ
authors.lastname: McCarthy
authors.forename: Rose
authors.initials: R
authors.lastname: Lai
authors.forename: Beatrice S
authors.initials: BS
authors.lastname: Melin
authors.forename: Melissa L
authors.initials: ML
authors.lastname: Bondy
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D002675:Child, Preschool; D003718:Denmark / Q000453:epidemiology; D005192:Family Health; D005260:Female; D005910:Glioma / Q000453:epidemiology / Q000235:genetics* / Q000473:pathology; D006801:Humans; D015994:Incidence; D007223:Infant; D007557:Israel / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D060787:Neoplasm Grading; D010375:Pedigree; D015995:Prevalence; D012737:Sex Factors; D013548:Sweden / Q000453:epidemiology; D014481:United States / Q000453:epidemiology; D055815:Young Adult