Ependymoma risk factors

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Ahmad Al Maradni, M.D. [2]


Common risk factors in the development of ependymoma are certain hereditary diseases (neurofibromatosis type II and Turcot syndrome), over-expression of kinetochore proteins, and down-regulation of metallothioneins.

Risk Factors

  • Children with certain hereditary diseases, such as neurofibromatosis type II (NF2), Turcot syndrome B, and MEN1 syndrome, have been found to be more frequently afflicted with ependymal tumors.
  • Increased occurrence of chromosome 1q and proteins such as tenascin C and epidermal growth factor are associated with increased risk for developing ependymal tumors.
  • ERBB2, ERBB4, and human telomerase reverse transcriptase TERT gene expression promote tumor cell proliferation, contributing to aggressive tumor behavior.
  • High expression of epidermal growth factor receptor EGFR correlates with unfavorable outcome.[1]
  • Over-expression of kinetochore proteins and down-regulation of metallothioneins are associated with recurrence of ependymomas.[2]


  1. Mendrzyk F, Korshunov A, Benner A, Toedt G, Pfister S, Radlwimmer B; et al. (2006). "Identification of gains on 1q and epidermal growth factor receptor overexpression as independent prognostic markers in intracranial ependymoma". Clin Cancer Res. 12 (7 Pt 1): 2070–9. doi:10.1158/1078-0432.CCR-05-2363. PMID 16609018.
  2. Peyre M, Commo F, Dantas-Barbosa C, Andreiuolo F, Puget S, Lacroix L; et al. (2010). "Portrait of ependymoma recurrence in children: biomarkers of tumor progression identified by dual-color microarray-based gene expression analysis". PLoS One. 5 (9): e12932. doi:10.1371/journal.pone.0012932. PMC 2945762. PMID 20885975.

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