Acta Neurochirurgica

, Volume 160, Issue 3, pp 439–447 | Cite as

Intramedullary ependymoma: long-term outcome after surgery

  • N. Svoboda
  • O. Bradac
  • P. de Lacy
  • V. Benes
Review Article - Spine



Overall (OS) and progression-free survival (PFS) of patients undergoing spinal ependymoma resection has been frequently reported. Contrarily, OS and PFS of purely intramedullary ependymomas have not been clearly determined yet.


The data of 37 patients undergoing resection of an intramedullary ependymoma (IE) from January 2000 to December 2016 were analysed retrospectively.


The mean age was 46 years. The male:female ratio was 24:13. The median duration of symptoms was 12 months. Sixty-two per cent of ependymomas were in the cervical, 24% in the thoracic, and 14% in the conus region in our series. The median volume was 1.3 ml. A syrinx was found in 49% and a cyst in 32%. GTR was achieved in 89%, STR in three (8%), and PR in one patient (3%). Median follow-up was 114 months. PFS was 87%, 82%, and 82% at 5, 10, and 15 years, respectively. OS was 97%, 88%, and 63% at 5, 10, and 15 years, respectively. There was a significant difference in PFS depending on the extent of resection and in OS depending on the pre-operative clinical status. There was no significant difference in OS and PFS regarding the other examined influencing factors.


GTR resection was the most important factor influencing PFS. According to our results OS of IEs is much worse than that of spinal ependymomas. Our analysis confirms that patients with good pre-operative (McCormick grade 1 and 2) clinical status have significantly better OS than patients with McCormick grade 3 and higher.


Intramedullary Ependymoma Spine tumour Prognosis 



No funding was received for this research.

Compliance with ethical standards

Conflicts of interests

All authors certify that they have no affiliationswith or involvement in any organisation or entity with any financial interest or non-financial interest in the subject matter or materials discussed in this manuscript.

Ethical approval

For this type of study formal consent is not required.


  1. 1.
    Abdullah KG, Lubelski D, Miller J, Steinmetz MP, Shin JH, Krishnaney A, Mroz TE, Benzel EC (2015) Progression free survival and functional outcome after surgical resection of intramedullary ependymomas. Journal of clinical neuroscience: official journal of the Neurosurgical Society of Australasia 22:1933–1937. CrossRefGoogle Scholar
  2. 2.
    Adam Y, Benezech J, Blanquet A, Fuentes JM, Bousigue JY, Debono B, Duplessis E, Espagno C, Plas JY, Lescure JP, Destandau J, Hladky JP, Grunewald P, Mahla K, Remond J, Louis E (2010) Intramedullary tumors. Results of a national investigation in private neurosurgery. Neuro-Chirurgie 56:344–349. CrossRefPubMedGoogle Scholar
  3. 3.
    Aghakhani N, David P, Parker F, Lacroix C, Benoudiba F, Tadie M (2008) Intramedullary spinal ependymomas: analysis of a consecutive series of 82 adult cases with particular attention to patients with no preoperative neurological deficit. Neurosurgery 62:1279–1285; discussion 1285-1276. CrossRefPubMedGoogle Scholar
  4. 4.
    Asazuma T, Toyama Y, Suzuki N, Fujimura Y, Hirabayshi K (1999) Ependymomas of the spinal cord and cauda equina: an analysis of 26 cases and a review of the literature. Spinal Cord 37:753–759CrossRefPubMedGoogle Scholar
  5. 5.
    Balasubramaniam S, Tyagi DK, Desai KI, Dighe MP (2016) Outcome analysis in cases of spinal conus cauda ependymoma. Journal of clinical and diagnostic research: JCDR 10:PC12–PC16. PubMedPubMedCentralGoogle Scholar
  6. 6.
    Bansal S, Ailawadhi P, Suri A, Kale SS, Sarat Chandra P, Singh M, Kumar R, Sharma BS, Mahapatra AK, Sharma MC, Sarkar C, Bithal P, Dash HH, Gaikwad S, Mishra NK (2013) Ten years' experience in the management of spinal intramedullary tumors in a single institution. Journal of clinical neuroscience: official journal of the Neurosurgical Society of Australasia 20:292–298. CrossRefGoogle Scholar
  7. 7.
    Benes V (2010) Intramedulární astrocytom—soubor 15 pacientů a přehled literatury. Cesk Slov Neurol N 73:169–177Google Scholar
  8. 8.
    Bostrom A, von Lehe M, Hartmann W, Pietsch T, Feuss M, Bostrom JP, Schramm J, Simon M (2011) Surgery for spinal cord ependymomas: outcome and prognostic factors. Neurosurgery 68:302–308; discussion 309. CrossRefPubMedGoogle Scholar
  9. 9.
    Brotchi J (2002) Intrinsic spinal cord tumor resection. Neurosurgery 50:1059–1063PubMedGoogle Scholar
  10. 10.
    Brotchi J, Bruneau M, Lefranc F, Baleriaux D (2006) Surgery of intraspinal cord tumors. Clin Neurosurg 53:209–216PubMedGoogle Scholar
  11. 11.
    Brotchi J, Fischer G (1998) Spinal cord ependymomas. Neurosurg Focus 4:e2CrossRefPubMedGoogle Scholar
  12. 12.
    Constantini S, Miller DC, Allen JC, Rorke LB, Freed D, Epstein FJ (2000) Radical excision of intramedullary spinal cord tumors: surgical morbidity and long-term follow-up evaluation in 164 children and young adults. J Neurosurg 93:183–193CrossRefPubMedGoogle Scholar
  13. 13.
    Cooper PR (1989) Outcome after operative treatment of intramedullary spinal cord tumors in adults: intermediate and long-term results in 51 patients. Neurosurgery 25:855–859CrossRefPubMedGoogle Scholar
  14. 14.
    Cooper PR, Epstein F (1985) Radical resection of intramedullary spinal cord tumors in adults. Recent experience in 29 patients. J Neurosurg 63:492–499. CrossRefPubMedGoogle Scholar
  15. 15.
    Cristante L, Herrmann HD (1994) Surgical management of intramedullary spinal cord tumors: functional outcome and sources of morbidity. Neurosurgery 35:69–74 discussion 74-66CrossRefPubMedGoogle Scholar
  16. 16.
    Dorr RT, Salmon SE, Robertone A, Bonnem E (1988) Phase I-II trial of interferon-alpha 2b by continuous subcutaneous infusion over 28 days. J Interf Res 8:717–725CrossRefGoogle Scholar
  17. 17.
    Epstein F, Epstein N (1981) Surgical management of holocord intramedullary spinal cord astrocytomas in children. J Neurosurg 54:829–832. CrossRefPubMedGoogle Scholar
  18. 18.
    Eroes CA, Zausinger S, Kreth FW, Goldbrunner R, Tonn JC (2010) Intramedullary low grade astrocytoma and ependymoma. Surgical results and predicting factors for clinical outcome. Acta Neurochir 152:611–618. CrossRefPubMedGoogle Scholar
  19. 19.
    Ferrante L, Mastronardi L, Celli P, Lunardi P, Acqui M, Fortuna A (1992) Intramedullary spinal cord ependymomas—a study of 45 cases with long-term follow-up. Acta Neurochir 119:74–79CrossRefPubMedGoogle Scholar
  20. 20.
    Garces-Ambrossi GL, McGirt MJ, Mehta VA, Sciubba DM, Witham TF, Bydon A, Wolinksy JP, Jallo GI, Gokaslan ZL (2009) Factors associated with progression-free survival and long-term neurological outcome after resection of intramedullary spinal cord tumors: analysis of 101 consecutive cases. Journal of neurosurgery Spine 11:591–599. CrossRefPubMedGoogle Scholar
  21. 21.
    Giammattei L, Penet N, Parker F, Messerer M (2016) Intramedullary ependymoma: microsurgical resection technique. Neuro-Chirurgie.
  22. 22.
    Gilbert MR, Ruda R, Soffietti R (2010) Ependymomas in adults. Current neurology and neuroscience reports 10:240–247. CrossRefPubMedGoogle Scholar
  23. 23.
    Gomez DR, Missett BT, Wara WM, Lamborn KR, Prados MD, Chang S, Berger MS, Haas-Kogan DA (2005) High failure rate in spinal ependymomas with long-term follow-up. Neuro-Oncology 7:254–259. CrossRefPubMedPubMedCentralGoogle Scholar
  24. 24.
    Green RM, Cloughesy TF, Stupp R, DeAngelis LM, Woyshner EA, Ney DE, Lassman AB (2009) Bevacizumab for recurrent ependymoma. Neurology 73:1677–1680. CrossRefPubMedPubMedCentralGoogle Scholar
  25. 25.
    Hejazi N, Hassler W (1998) Microsurgical treatment of intramedullary spinal cord tumors. Neurol Med Chir 38:266–271 discussion 271-263CrossRefGoogle Scholar
  26. 26.
    Hoshimaru M, Koyama T, Hashimoto N, Kikuchi H (1999) Results of microsurgical treatment for intramedullary spinal cord ependymomas: analysis of 36 cases. Neurosurgery 44:264–269CrossRefPubMedGoogle Scholar
  27. 27.
    Chamberlain MC (2002) Salvage chemotherapy for recurrent spinal cord ependymona. Cancer 95:997–1002. CrossRefPubMedGoogle Scholar
  28. 28.
    Chang UK, Choe WJ, Chung SK, Chung CK, Kim HJ (2002) Surgical outcome and prognostic factors of spinal intramedullary ependymomas in adults. J Neuro-Oncol 57:133–139CrossRefGoogle Scholar
  29. 29.
    Isaacson SR (2000) Radiation therapy and the management of intramedullary spinal cord tumors. J Neuro-Oncol 47:231–238CrossRefGoogle Scholar
  30. 30.
    Iunes EA, Stavale JN, de Cassia Caldas Pessoa R, Ansai R, Onishi FJ, de Paiva Neto MA, de Padua BA, Cavalheiro S, Fleury Malheiros SM (2011) Multifocal intradural extramedullary ependymoma. Case report. Journal of neurosurgery Spine 14:65–70. CrossRefPubMedGoogle Scholar
  31. 31.
    Iwasaki Y, Hida K, Sawamura Y, Abe H (2000) Spinal intramedullary ependymomas: surgical results and immunohistochemical analysis of tumour proliferation activity. Br J Neurosurg 14:331–336CrossRefPubMedGoogle Scholar
  32. 32.
    Kane PJ, El-Mahdy W, Singh A, Powell MP, Crockard HA (1999) Spinal intradural tumours: part II—Intramedullary. Br J Neurosurg 13:558–563CrossRefPubMedGoogle Scholar
  33. 33.
    Karikari IO, Nimjee SM, Hodges TR, Cutrell E, Hughes BD, Powers CJ, Mehta AI, Hardin C, Bagley CA, Isaacs RE, Haglund MM, Friedman AH (2015) Impact of tumor histology on resectability and neurological outcome in primary intramedullary spinal cord tumors: a single-center experience with 102 patients. Neurosurgery 76(Suppl 1):S4–13; discussion S13. CrossRefPubMedGoogle Scholar
  34. 34.
    Klekamp J (2013) Treatment of intramedullary tumors: analysis of surgical morbidity and long-term results. Journal of neurosurgery Spine 19:12–26. CrossRefPubMedGoogle Scholar
  35. 35.
    Klekamp J (2015) Spinal ependymomas. Part 1: Intramedullary ependymomas. Neurosurg Focus 39:E6. CrossRefPubMedGoogle Scholar
  36. 36.
    Kucia EJ, Bambakidis NC, Chang SW, Spetzler RF (2011) Surgical technique and outcomes in the treatment of spinal cord ependymomas, part 1: intramedullary ependymomas. Neurosurgery 68:57–63; discussion 63. PubMedGoogle Scholar
  37. 37.
    Lee TT, Gromelski EB, Green BA (1998) Surgical treatment of spinal ependymoma and post-operative radiotherapy. Acta Neurochir 140:309–313CrossRefPubMedGoogle Scholar
  38. 38.
    Manzano G, Green BA, Vanni S, Levi AD (2008) Contemporary management of adult intramedullary spinal tumors-pathology and neurological outcomes related to surgical resection. Spinal Cord 46:540–546. CrossRefPubMedGoogle Scholar
  39. 39.
    Matsuyama Y, Sakai Y, Katayama Y, Imagama S, Ito Z, Wakao N, Sato K, Kamiya M, Yukawa Y, Kanemura T, Yanase M, Ishiguro N (2009) Surgical results of intramedullary spinal cord tumor with spinal cord monitoring to guide extent of resection. Journal of neurosurgery Spine 10:404–413. CrossRefPubMedGoogle Scholar
  40. 40.
    McCormick PC, Torres R, Post KD, Stein BM (1990) Intramedullary ependymoma of the spinal cord. J Neurosurg 72:523–532. CrossRefPubMedGoogle Scholar
  41. 41.
    McGuire CS, Sainani KL, Fisher PG (2009) Incidence patterns for ependymoma: a surveillance, epidemiology, and end results study. J Neurosurg 110:725–729. CrossRefPubMedGoogle Scholar
  42. 42.
    Merchant TE, Fouladi M (2005) Ependymoma: new therapeutic approaches including radiation and chemotherapy. J Neuro-Oncol 75:287–299. CrossRefGoogle Scholar
  43. 43.
    Nagasawa DT, Smith ZA, Cremer N, Fong C, DC L, Yang I (2011) Complications associated with the treatment for spinal ependymomas. Neurosurg Focus 31:E13. CrossRefPubMedGoogle Scholar
  44. 44.
    Oh MC, Ivan ME, Sun MZ, Kaur G, Safaee M, Kim JM, Sayegh ET, Aranda D, Parsa AT (2013) Adjuvant radiotherapy delays recurrence following subtotal resection of spinal cord ependymomas. Neuro-Oncology 15:208–215. CrossRefPubMedGoogle Scholar
  45. 45.
    Oh MC, Kim JM, Kaur G, Safaee M, Sun MZ, Singh A, Aranda D, Molinaro AM, Parsa AT (2013) Prognosis by tumor location in adults with spinal ependymomas. Journal of neurosurgery Spine 18:226–235. CrossRefPubMedGoogle Scholar
  46. 46.
    Ohata K, Takami T, Gotou T, El-Bahy K, Morino M, Maeda M, Inoue Y, Hakuba A (1999) Surgical outcome of intramedullary spinal cord ependymoma. Acta Neurochir 141:341–346 discussion 346-347CrossRefPubMedGoogle Scholar
  47. 47.
    Prokopienko M, Kunert P, Podgorska A, Marchel A (2017) Surgical treatment of intramedullary ependymomas. Neurol Neurochir Pol.
  48. 48.
    Samii M, Klekamp J (1994) Surgical results of 100 intramedullary tumors in relation to accompanying syringomyelia. Neurosurgery 35:865–873 discussion 873CrossRefPubMedGoogle Scholar
  49. 49.
    Sandalcioglu IE, Gasser T, Asgari S, Lazorisak A, Engelhorn T, Egelhof T, Stolke D, Wiedemayer H (2005) Functional outcome after surgical treatment of intramedullary spinal cord tumors: experience with 78 patients. Spinal Cord 43:34–41. CrossRefPubMedGoogle Scholar
  50. 50.
    Sgouros S, Malluci CL, Jackowski A (1996) Spinal ependymomas—the value of postoperative radiotherapy for residual disease control. Br J Neurosurg 10:559–566CrossRefPubMedGoogle Scholar
  51. 51.
    Shaw EG, Evans RG, Scheithauer BW, Ilstrup DM, Earle JD (1986) Radiotherapeutic management of adult intraspinal ependymomas. Int J Radiat Oncol Biol Phys 12:323–327CrossRefPubMedGoogle Scholar
  52. 52.
    Schild SE, Nisi K, Scheithauer BW, Wong WW, Lyons MK, Schomberg PJ, Shaw EG (1998) The results of radiotherapy for ependymomas: the Mayo Clinic experience. Int J Radiat Oncol Biol Phys 42:953–958CrossRefPubMedGoogle Scholar
  53. 53.
    Schwartz TH, McCormick PC (2000) Intramedullary ependymomas: clinical presentation, surgical treatment strategies and prognosis. J Neuro-Oncol 47:211–218CrossRefGoogle Scholar
  54. 54.
    Sweeney KJ, Reynolds M, Farrell M, Bolger C (2016) Gross total resection rates of grade II/III intramedullary ependymomas using the surgical strategy of en-bloc resection without intra-operative neurophysiological monitoring. Br J Neurosurg 1–5.
  55. 55.
    Tan CT, Hancock CH, Mondora A, Hoffman NW (1984) Phase I study of aziridinylbenzoquinone (AZQ, NSC 182986) in children with cancer. Cancer Res 44:831–835PubMedGoogle Scholar
  56. 56.
    Taylor RE (2004) Review of radiotherapy dose and volume for intracranial ependymoma. Pediatr Blood Cancer 42:457–460. CrossRefPubMedGoogle Scholar
  57. 57.
    Whitaker SJ, Bessell EM, Ashley SE, Bloom HJ, Bell BA, Brada M (1991) Postoperative radiotherapy in the management of spinal cord ependymoma. J Neurosurg 74:720–728. CrossRefPubMedGoogle Scholar
  58. 58.
    Xu QW, Bao WM, Mao RL, Yang GY (1996) Aggressive surgery for intramedullary tumor of cervical spinal cord. Surg Neurol 46:322–328CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag GmbH Austria, part of Springer Nature 2017

Authors and Affiliations

  1. 1.Department of Neurosurgery and NeurooncologyMilitary University Hospital and Charles University, First Medical FacultyPrague 6Czech Republic

Personalised recommendations