Skip to main content
SpringerLink
Log in

Spinal Cord Ependymoma

  • Chapter
  • First Online:
Adult CNS Radiation Oncology

  • 1989 Accesses

Abstract

Ependymomas of the spinal cord are glial tumors arising from ependymal cells along the central canal of the spinal cord. They are rare, typically have a long natural history, and behave differently than their intracranial counterparts. They are grouped based on the World Health Organization (WHO) classification from grade I to III, with grade II spinal cord ependymomas appearing to be the most indolent despite their intermediate grade. Myxopapillary ependymoma (MPE) is a distinct subset that occurs almost exclusively in the conus medullaris, cauda equina, or filum terminale. Spinal cord ependymomas often present insidiously with back pain or neurologic deficits that are dependent on their location within the cord. Management consists of surgical resection, with gross total resection (GTR) offering the best chance for long-term control and possible cure. The role of postoperative radiation is controversial, but in general, radiation is employed locally for subtotal resection (STR), grade III (anaplastic) ependymomas, or after re-resection for recurrent disease. Long-term survival is excellent with rates over 85% at 10 years. However, 17 to 30% will recur, often years following initial treatment. As such, continued follow-up with surveillance imaging is essential.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 139.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 179.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 249.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Villano JL, Parker CK, Dolecek TA. Descriptive epidemiology of ependymal tumours in the United States. Br J Cancer. 2013;108(11):2367–71.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  2. Vera-Bolanos E, Aldape K, Yuan Y, Wu J, et al. Clinical course and progression-free survival of adult intracranial and spinal ependymoma patients. Neuro Oncol. 2015;17(3):440–7.

    Article  PubMed  Google Scholar 

  3. Ostrom QT, Gittleman H, Fulop J, et al. CBTRUS statistical report: primary brain and central nervous system tumors diagnosed in the United States in 2008–2012. Neuro Oncol. 2015;17(Suppl 4):iv1–iv62.

    Article  PubMed  PubMed Central  Google Scholar 

  4. Lee SH, Chung CK, Kim CH, et al. Long-term outcomes of surgical resection with or without adjuvant radiation therapy for treatment of spinal ependymoma: a retrospective multicenter study by the Korea Spinal Oncology Research Group. Neuro Oncol. 2013;15(7):921–9.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  5. Oh MC, Ivan ME, Sun MZ, et al. Adjuvant radiotherapy delays recurrence following subtotal resection of spinal cord ependymomas. Neuro Oncol. 2013;15(2):208–15.

    Article  CAS  PubMed  Google Scholar 

  6. Tarapore PE, Modera P, Naujokas A, et al. Pathology of spinal ependymomas: an institutional experience over 25 years in 134 patients. Neurosurgery. 2013;73(2):247–55. discussion 55

    Article  PubMed  Google Scholar 

  7. Bates JE, Choi G, Milano MT. Myxopapillary ependymoma: a SEER analysis of epidemiology and outcomes. J Neurooncol. 2016;129(2):251–8.

    Article  PubMed  Google Scholar 

  8. Weber DC, Wang Y, Miller R, et al. Long-term outcome of patients with spinal myxopapillary ependymoma: treatment results from the MD Anderson Cancer Center and institutions from the Rare Cancer Network. Neuro Oncol. 2015;17(4):588–95.

    Article  PubMed  Google Scholar 

  9. Mautner VF, Tatagiba M, Lindenau M, et al. Spinal tumors in patients with neurofibromatosis type 2: MR imaging study of frequency, multiplicity, and variety. AJR Am J Roentgenol. 1995;165(4):951–5.

    Article  CAS  PubMed  Google Scholar 

  10. Patronas NJ, Courcoutsakis N, Bromley CM, et al. Intramedullary and spinal canal tumors in patients with neurofibromatosis 2: MR imaging findings and correlation with genotype. Radiology. 2001;218(2):434–42.

    Article  CAS  PubMed  Google Scholar 

  11. Plotkin SR, O’Donnell CC, Curry WT, et al. Spinal ependymomas in neurofibromatosis type 2: a retrospective analysis of 55 patients. J Neurosurg Spine. 2011;14(4):543–7.

    Article  PubMed  Google Scholar 

  12. Acquaye AA, Vera E, Gilbert MR, et al. Clinical presentation and outcomes for adult ependymoma patients. Cancer. 2017;123(3):494–501.

    Article  PubMed  Google Scholar 

  13. Oh MC, Kim JM, Kaur G, et al. Prognosis by tumor location in adults with spinal ependymomas. J Neurosurg Spine. 2013;18(3):226–35.

    Article  PubMed  Google Scholar 

  14. Louis DN, Perry A, Reifenberger et al. The 2016 World Health Organization classification of tumors of the central nervous system: a summary. Acta Neuropathol. 2016;131(6):803–20.

    Google Scholar 

  15. Wu J, Armstrong TS, Gilbert MR. Biology and management of ependymomas. Neuro Oncol. 2016;18(7):902–13.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  16. Waldron JN, Laperriere NJ, Jaakkimainen L, Simpson WJ, Payne D, Milosevic M, et al. Spinal cord ependymomas: a retrospective analysis of 59 cases. Int J Radiat Oncol Biol Phys. 1993;27(2):223–9.

    Article  CAS  PubMed  Google Scholar 

  17. Mork SJ, Loken AC. Ependymoma: a follow-up study of 101 cases. Cancer. 1977;40(2):907–15.

    Article  CAS  PubMed  Google Scholar 

  18. Armstrong TS, Vera-Bolanos E, Bekele BN, et al. Adult ependymal tumors: prognosis and the M. D. Anderson Cancer Center experience. Neuro Oncol. 2010;12(8):862–70.

    Article  PubMed  PubMed Central  Google Scholar 

  19. Wahab SH, Simpson JR, Michalski JM, et al. Long term outcome with post-operative radiation therapy for spinal canal ependymoma. J Neurooncol. 2007;83(1):85–9.

    Article  PubMed  Google Scholar 

  20. Cervoni L, Celli P, Fortuna A, et al. Recurrence of spinal ependymoma. Risk factors and long-term survival. Spine (Phila Pa 1976). 1994;19(24):2838–41.

    Article  CAS  Google Scholar 

  21. Hanbali F, Fourney DR, Marmor E, et al. Spinal cord ependymoma: radical surgical resection and outcome. Neurosurgery. 2002;51(5):1162–72. discussion 72–4

    Article  PubMed  Google Scholar 

  22. Marks JE, Adler SJ. A comparative study of ependymomas by site of origin. Int J Radiat Oncol Biol Phys. 1982;8(1):37–43.

    Article  CAS  PubMed  Google Scholar 

  23. Abdel-Wahab M, Etuk B, Palermo J, et al. Spinal cord gliomas: a multi-institutional retrospective analysis. Int J Radiat Oncol Biol Phys. 2006;64(4):1060–71.

    Article  PubMed  Google Scholar 

  24. Lee CH, Chung CK, Ohn JH, et al. The similarities and differences between intracranial and spinal ependymomas: a review from a genetic research perspective. J Korean Neurosurg Soc. 2016;59(2):83–90.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  25. Lee CH, Chung CK, Kim CH. Genetic differences on intracranial versus spinal cord ependymal tumors: a meta-analysis of genetic researches. Eur Spine J. 2016;25(12):3942–51.

    Article  PubMed  Google Scholar 

  26. Raghunathan A, Wani K, Armstrong TS, et al. Histological predictors of outcome in ependymoma are dependent on anatomic site within the central nervous system. Brain Pathol. 2013;23(5):584–94.

    Article  PubMed  Google Scholar 

  27. Gomez DR, Missett BT, Wara WM, et al. High failure rate in spinal ependymomas with long-term follow-up. Neuro Oncol. 2005;7(3):254–9.

    Article  PubMed  PubMed Central  Google Scholar 

  28. Shaw EG, Evans RG, Scheithauer BW, et al. Radiotherapeutic management of adult intraspinal ependymomas. Int J Radiat Oncol Biol Phys. 1986;12(3):323–7.

    Article  CAS  PubMed  Google Scholar 

  29. Kotecha R, Modugula S, Angelov L, et al. The role of adjuvant radiation therapy in patients with myxopapillary ependymomas. Int J Radiat Oncol Biol Phys. 2016;96((2):E112.

    Article  Google Scholar 

  30. Klekamp J. Spinal ependymomas. Part 2: Ependymomas of the filum terminale. Neurosurg Focus. 2015;39(2):E7.

    Article  PubMed  Google Scholar 

  31. Sonneland PR, Scheithauer BW, Onofrio BM. Myxopapillary ependymoma. A clinicopathologic and immunocytochemical study of 77 cases. Cancer. 1985;56(4):883–93.

    Article  CAS  PubMed  Google Scholar 

  32. Chao ST, Kobayashi T, Benzel E, et al. The role of adjuvant radiation therapy in the treatment of spinal myxopapillary ependymomas. J Neurosurg Spine. 2011;14(1):59–64.

    Article  PubMed  Google Scholar 

  33. Abdulaziz M, Mallory GW, Bydon M, et al. Outcomes following myxopapillary ependymoma resection: the importance of capsule integrity. Neurosurg Focus. 2015;39(2):E8.

    Article  PubMed  Google Scholar 

  34. Akyurek S, Chang EL, Yu TK, et al. Spinal myxopapillary ependymoma outcomes in patients treated with surgery and radiotherapy at M.D. Anderson Cancer Center. J Neurooncol. 2006;80(2):177–83.

    Article  PubMed  Google Scholar 

  35. Tsai CJ, Wang Y, Allen PK, et al. Outcomes after surgery and radiotherapy for spinal myxopapillary ependymoma: update of the MD Anderson Cancer Center experience. Neurosurgery. 2014;75(3):205–14. discussion 13–4

    Article  PubMed  Google Scholar 

  36. Pica A, Miller R, Villa S, et al. The results of surgery, with or without radiotherapy, for primary spinal myxopapillary ependymoma: a retrospective study from the rare cancer network. Int J Radiat Oncol Biol Phys. 2009;74(4):1114–20.

    Article  PubMed  Google Scholar 

  37. Schultheiss TE, Kun LE, Ang KK, et al. Radiation response of the central nervous system. Int J Radiat Oncol Biol Phys. 1995;31(5):1093–112.

    Article  CAS  PubMed  Google Scholar 

  38. Pieters RS, Niemierko A, Fullerton BC, et al. Cauda equina tolerance to high-dose fractionated irradiation. Int J Radiat Oncol Biol Phys. 2006;64(1):251–7.

    Article  PubMed  Google Scholar 

  39. Fein DA, Marcus RB Jr, Parsons JT, et al. Lhermitte’s sign: incidence and treatment variables influencing risk after irradiation of the cervical spinal cord. Int J Radiat Oncol Biol Phys. 1993;27(5):1029–33.

    Article  CAS  PubMed  Google Scholar 

  40. Feldman WB, Clark AJ, Safaee M, et al. Tumor control after surgery for spinal myxopapillary ependymomas: distinct outcomes in adults versus children: a systematic review. J Neurosurg Spine. 2013;19(4):471–6.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Radiation Oncology, Taussig Cancer Institute, Cleveland Clinic, Cleveland, OH, USA

    Martin C. Tom, Ehsan H. Balagamwala, John H. Suh & Samuel T. Chao

  2. Rose Ella Burkhardt Brain Tumor and Neuro-oncology Center, Cleveland Clinic, Cleveland, OH, USA

    John H. Suh & Samuel T. Chao

Authors
  1. Martin C. Tom
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Ehsan H. Balagamwala
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. John H. Suh
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Samuel T. Chao
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Samuel T. Chao .

Editor information

Editors and Affiliations

  1. Department of Radiation Oncology, Keck School of Medicine of USC, Norris Cancer Hospital, Los Angeles, California, USA

    Eric L. Chang

  2. Department of Radiation Oncology, Mayo Clinic, Rochester, Minnesota, USA

    Paul D. Brown

  3. Department of Radiation Oncology, University of Washington Medical Center, Seattle, Washington, USA

    Simon S. Lo

  4. Department of Radiation Oncology, Sunnybrook Health Sciences Centre, University of Toronto, Toronto, Ontario, Canada

    Arjun Sahgal

  5. Department of Radiation Oncology, Taussig Cancer Institute Cleveland Clinic, Cleveland, Ohio, USA

    John H. Suh

Rights and permissions

Reprints and permissions

Copyright information

© 2018 Springer International Publishing AG, part of Springer Nature

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Tom, M.C., Balagamwala, E.H., Suh, J.H., Chao, S.T. (2018). Spinal Cord Ependymoma. In: Chang, E., Brown, P., Lo, S., Sahgal, A., Suh, J. (eds) Adult CNS Radiation Oncology. Springer, Cham. https://doi.org/10.1007/978-3-319-42878-9_10

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-42878-9_10

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-42877-2

  • Online ISBN: 978-3-319-42878-9

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation