Ependymoma natural history

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Ependymoma Microchapters


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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]


If left untreated, patients with ependymoma may progress to develop nausea, vomiting, headache, and irritability. Common complications of ependymoma include seizure, hydrocephalus, muscle paralysis, and speech problems.


Common complications associated with ependymomas are:


Unfavorable factors affecting outcome include the following:[1]

  • Gain of chromosome 1q25 is present in approximately 20% of pediatric intracranial ependymoma cases and has been reported as a negative prognostic factor by multiple research groups.[2][3]
  • Gene expression profile[4]
  • Other factors that have been reported to be associated with poor prognosis for pediatric ependymoma include expression of the enzymatic subunit of telomerase (hTERT) and expression of the neural stem cell marker Nestin.[5][6]
  • Tumor location. Cranial variants of ependymoma have a less favorable outcome than primary spinal cord ependymomas. Location within the spinal cord may also affect outcome, with tumors in the lower portion of the spinal cord having a worse prognosis.[7]
  • Younger age at diagnosis.[8]
  • Anaplastic histology[9]
  • Subtotal resection[10]
  • Lower doses of radiation[11]
  • Immunohistochemical testing has identified increased expression of markers of proliferation (e.g., Ki-67 and MIB-1) and increased expression of EZH2, a polycomb complex protein involved in epigenetic regulation of gene expression, as prognostic factors for greater risk of treatment failure.[12][13][14]


  1. Eoendymoma. http://www.cancer.gov/types/brain/hp/child-ependymoma-treatment-pdq#section/_35 URL Accessed on 10 6 2015.
  2. Godfraind C, Kaczmarska JM, Kocak M, Dalton J, Wright KD, Sanford RA et al. (2012). "Distinct disease-risk groups in pediatric supratentorial and posterior fossa ependymomas.". Acta Neuropathol 124 (2): 247-57. doi:10.1007/s00401-012-0981-9. PMID 22526017.
  3. 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.
  4. Wani K, Armstrong TS, Vera-Bolanos E, Raghunathan A, Ellison D, Gilbertson R et al. (2012). "A prognostic gene expression signature in infratentorial ependymoma.". Acta Neuropathol 123 (5): 727-38. doi:10.1007/s00401-012-0941-4. PMID 22322993.
  5. Tabori U, Ma J, Carter M, Zielenska M, Rutka J, Bouffet E et al. (2006). "Human telomere reverse transcriptase expression predicts progression and survival in pediatric intracranial ependymoma.". J Clin Oncol 24 (10): 1522-8. doi:10.1200/JCO.2005.04.2127. PMID 16575002.
  6. Modena P, Buttarelli FR, Miceli R, Piccinin E, Baldi C, Antonelli M et al. (2012). "Predictors of outcome in an AIEOP series of childhood ependymomas: a multifactorial analysis.". Neuro Oncol 14 (11): 1346-56. doi:10.1093/neuonc/nos245. PMID 23076205.
  7. Oh MC, Sayegh ET, Safaee M, Sun MZ, Kaur G, Kim JM et al. (2013). "Prognosis by tumor location for pediatric spinal cord ependymomas.". J Neurosurg Pediatr 11 (3): 282-8. doi:10.3171/2012.11.PEDS12292. PMID 23259510.
  8. Tamburrini G, D'Ercole M, Pettorini BL, Caldarelli M, Massimi L, Di Rocco C (2009). "Survival following treatment for intracranial ependymoma: a review.". Childs Nerv Syst 25 (10): 1303-12. doi:10.1007/s00381-009-0874-y. PMID 19387655.
  9. Korshunov A, Golanov A, Sycheva R, Timirgaz V (2004). "The histologic grade is a main prognostic factor for patients with intracranial ependymomas treated in the microneurosurgical era: an analysis of 258 patients.". Cancer 100 (6): 1230-7. doi:10.1002/cncr.20075. PMID 15022291.
  10. White F (1975). "Epidemiology and infection control.". Dimens Health Serv 52 (12): 34, 37, 39. PMID 1303-12.
  11. Vaidya K, Smee R, Williams JR (2012). "Prognostic factors and treatment options for paediatric ependymomas.". J Clin Neurosci 19 (9): 1228-35. doi:10.1016/j.jocn.2012.02.006. PMID 22840355.
  12. Li AM, Dunham C, Tabori U, Carret AS, McNeely PD, Johnston D et al. (2015). "EZH2 expression is a prognostic factor in childhood intracranial ependymoma: a Canadian Pediatric Brain Tumor Consortium study.". Cancer 121 (9): 1499-507. doi:10.1002/cncr.29198. PMID 25586788.
  13. Wolfsberger S, Fischer I, Höftberger R, Birner P, Slavc I, Dieckmann K et al. (2004). "Ki-67 immunolabeling index is an accurate predictor of outcome in patients with intracranial ependymoma.". Am J Surg Pathol 28 (7): 914-20. PMID 15223962.
  14. Kurt E, Zheng PP, Hop WC, van der Weiden M, Bol M, van den Bent MJ et al. (2006). "Identification of relevant prognostic histopathologic features in 69 intracranial ependymomas, excluding myxopapillary ependymomas and subependymomas.". Cancer 106 (2): 388-95. doi:10.1002/cncr.21608. PMID 16342252.