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Spinal cord ependymoma

MedGen UID:
65968
•Concept ID:
C0238432
•
Neoplastic Process
Synonyms: ependymal neoplasm of the spinal cord; Ependymal tumor of spinal cord; Ependymoma of spinal cord; Ependymoma of Spinal Cord; ependymoma of spinal cord; Ependymoma of the Spinal Cord; ependymoma of the spinal cord; Spinal Cord Ependymoma; spinal cord ependymoma; Spinal Cord Ependymoma without MYCN Amplification; Spinal Ependymoma
SNOMED CT: Ependymal tumor of spinal cord (254949006); Ependymoma of spinal cord (254949006)
 
Monarch Initiative: MONDO:0003473

Definition

An ependymoma that arises from the spinal cord. It is characterized by the absence of MYCN gene amplification and frequent loss of chromosome 22q and mutations of NF2. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSpinal cord ependymoma

Professional guidelines

PubMed

Bicentric Treatment Outcomes After Proton Therapy for Nonmyxopapillary High-Grade Spinal Cord Ependymoma in Children.
Indelicato DJ, Ioakeim-Ioannidou M, Grippin AJ, Bradley JA, Mailhot Vega RB, Viviers E, Tarbell NJ, Yock TI, MacDonald SM
Int J Radiat Oncol Biol Phys 2022 Feb 1;112(2):335-341. Epub 2021 Sep 29 doi: 10.1016/j.ijrobp.2021.09.030. PMID: 34597719
Natural history of intramedullary spinal cord ependymoma in patients preferring nonoperative treatment.
Behmanesh B, Gessler F, Dützmann S, Dubinski D, Imoehl L, Seifert V, Setzer M, Marquardt G
J Neurooncol 2017 Oct;135(1):93-98. Epub 2017 Jun 30 doi: 10.1007/s11060-017-2552-3. PMID: 28667596
Treatment of pediatric Grade II spinal ependymomas: a population-based study.
Lin Y, Jea A, Melkonian SC, Lam S
J Neurosurg Pediatr 2015 Mar;15(3):243-9. Epub 2014 Dec 19 doi: 10.3171/2014.9.PEDS1473. PMID: 25525932

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Central Nervous System Cancers, 2023

Recent clinical studies

Etiology

Multisegmental versus monosegmental intramedullary spinal cord ependymomas: perioperative neurological functions and surgical outcomes.
Yang C, Sun J, Xie J, Ma C, Liu B, Wang T, Chen X, Wu J, Wu H, Zheng M, Chang Q, Yang J
Neurosurg Rev 2022 Feb;45(1):553-560. Epub 2021 May 14 doi: 10.1007/s10143-021-01567-5. PMID: 33988802
Postoperative Syrinx Shrinkage in Spinal Ependymoma of WHO Grade II.
Kobayashi K, Ando K, Machino M, Tanaka S, Morozumi M, Kanbara S, Ito S, Inoue T, Matsuyama Y, Ishiguro N, Imagama S
Clin Spine Surg 2021 Mar 1;34(2):E100-E106. doi: 10.1097/BSD.0000000000001061. PMID: 33633066
MRI characteristics and resectability in spinal cord glioma.
Seaman SC, Bathla G, Park BJ, Woodroffe RW, Smith M, Menezes AH, Noeller J, Yamaguchi S, Hitchon PW
Clin Neurol Neurosurg 2021 Jan;200:106321. Epub 2020 Oct 19 doi: 10.1016/j.clineuro.2020.106321. PMID: 33268194
A phase II study of dose-dense temozolomide and lapatinib for recurrent low-grade and anaplastic supratentorial, infratentorial, and spinal cord ependymoma.
Gilbert MR, Yuan Y, Wu J, Mendoza T, Vera E, Omuro A, Lieberman F, Robins HI, Gerstner ER, Wu J, Wen PY, Mikkelsen T, Aldape K, Armstrong TS
Neuro Oncol 2021 Mar 25;23(3):468-477. doi: 10.1093/neuonc/noaa240. PMID: 33085768Free PMC Article
Spinal cord ependymoma: a review of the literature and case series of ten patients.
Celano E, Salehani A, Malcolm JG, Reinertsen E, Hadjipanayis CG
J Neurooncol 2016 Jul;128(3):377-86. Epub 2016 May 6 doi: 10.1007/s11060-016-2135-8. PMID: 27154165Free PMC Article

Diagnosis

MRI characteristics and resectability in spinal cord glioma.
Seaman SC, Bathla G, Park BJ, Woodroffe RW, Smith M, Menezes AH, Noeller J, Yamaguchi S, Hitchon PW
Clin Neurol Neurosurg 2021 Jan;200:106321. Epub 2020 Oct 19 doi: 10.1016/j.clineuro.2020.106321. PMID: 33268194
Intramedullary spinal cord ependymoma and astrocytoma: intraoperative frozen-section diagnosis, extent of resection, and outcomes.
Hongo H, Takai K, Komori T, Taniguchi M
J Neurosurg Spine 2018 Oct 19;30(1):133-139. doi: 10.3171/2018.7.SPINE18230. PMID: 30485241
Spinal cord ependymoma: a review of the literature and case series of ten patients.
Celano E, Salehani A, Malcolm JG, Reinertsen E, Hadjipanayis CG
J Neurooncol 2016 Jul;128(3):377-86. Epub 2016 May 6 doi: 10.1007/s11060-016-2135-8. PMID: 27154165Free PMC Article
Spinal cord ependymoma in children--results of postoperative radiotherapy.
Pędziwiatr K, Skowrońska-Gardas A, Chojnacka M
Radiother Oncol 2013 Feb;106(2):181-5. Epub 2013 Mar 13 doi: 10.1016/j.radonc.2013.02.007. PMID: 23490267
Ependymoma of the spinal cord in children and adolescents: a retrospective series from the HIT database.
Benesch M, Weber-Mzell D, Gerber NU, von Hoff K, Deinlein F, Krauss J, Warmuth-Metz M, Kortmann RD, Pietsch T, Driever PH, Quehenberger F, Urban C, Rutkowski S
J Neurosurg Pediatr 2010 Aug;6(2):137-44. doi: 10.3171/2010.5.PEDS09553. PMID: 20672934

Therapy

Bicentric Treatment Outcomes After Proton Therapy for Nonmyxopapillary High-Grade Spinal Cord Ependymoma in Children.
Indelicato DJ, Ioakeim-Ioannidou M, Grippin AJ, Bradley JA, Mailhot Vega RB, Viviers E, Tarbell NJ, Yock TI, MacDonald SM
Int J Radiat Oncol Biol Phys 2022 Feb 1;112(2):335-341. Epub 2021 Sep 29 doi: 10.1016/j.ijrobp.2021.09.030. PMID: 34597719
A phase II study of dose-dense temozolomide and lapatinib for recurrent low-grade and anaplastic supratentorial, infratentorial, and spinal cord ependymoma.
Gilbert MR, Yuan Y, Wu J, Mendoza T, Vera E, Omuro A, Lieberman F, Robins HI, Gerstner ER, Wu J, Wen PY, Mikkelsen T, Aldape K, Armstrong TS
Neuro Oncol 2021 Mar 25;23(3):468-477. doi: 10.1093/neuonc/noaa240. PMID: 33085768Free PMC Article
Complications associated with the treatment for spinal ependymomas.
Nagasawa DT, Smith ZA, Cremer N, Fong C, Lu DC, Yang I
Neurosurg Focus 2011 Oct;31(4):E13. doi: 10.3171/2011.7.FOCUS11158. PMID: 21961857
Ependymoma of the spinal cord in children and adolescents: a retrospective series from the HIT database.
Benesch M, Weber-Mzell D, Gerber NU, von Hoff K, Deinlein F, Krauss J, Warmuth-Metz M, Kortmann RD, Pietsch T, Driever PH, Quehenberger F, Urban C, Rutkowski S
J Neurosurg Pediatr 2010 Aug;6(2):137-44. doi: 10.3171/2010.5.PEDS09553. PMID: 20672934
Brain tumors across the age spectrum: biology, therapy, and late effects.
Merchant TE, Pollack IF, Loeffler JS
Semin Radiat Oncol 2010 Jan;20(1):58-66. doi: 10.1016/j.semradonc.2009.09.005. PMID: 19959032Free PMC Article

Prognosis

Multisegmental versus monosegmental intramedullary spinal cord ependymomas: perioperative neurological functions and surgical outcomes.
Yang C, Sun J, Xie J, Ma C, Liu B, Wang T, Chen X, Wu J, Wu H, Zheng M, Chang Q, Yang J
Neurosurg Rev 2022 Feb;45(1):553-560. Epub 2021 May 14 doi: 10.1007/s10143-021-01567-5. PMID: 33988802
MRI characteristics and resectability in spinal cord glioma.
Seaman SC, Bathla G, Park BJ, Woodroffe RW, Smith M, Menezes AH, Noeller J, Yamaguchi S, Hitchon PW
Clin Neurol Neurosurg 2021 Jan;200:106321. Epub 2020 Oct 19 doi: 10.1016/j.clineuro.2020.106321. PMID: 33268194
Natural history of intramedullary spinal cord ependymoma in patients preferring nonoperative treatment.
Behmanesh B, Gessler F, Dützmann S, Dubinski D, Imoehl L, Seifert V, Setzer M, Marquardt G
J Neurooncol 2017 Oct;135(1):93-98. Epub 2017 Jun 30 doi: 10.1007/s11060-017-2552-3. PMID: 28667596
Spinal cord ependymoma: a review of the literature and case series of ten patients.
Celano E, Salehani A, Malcolm JG, Reinertsen E, Hadjipanayis CG
J Neurooncol 2016 Jul;128(3):377-86. Epub 2016 May 6 doi: 10.1007/s11060-016-2135-8. PMID: 27154165Free PMC Article
Ependymoma of the spinal cord in children and adolescents: a retrospective series from the HIT database.
Benesch M, Weber-Mzell D, Gerber NU, von Hoff K, Deinlein F, Krauss J, Warmuth-Metz M, Kortmann RD, Pietsch T, Driever PH, Quehenberger F, Urban C, Rutkowski S
J Neurosurg Pediatr 2010 Aug;6(2):137-44. doi: 10.3171/2010.5.PEDS09553. PMID: 20672934

Clinical prediction guides

Multisegmental versus monosegmental intramedullary spinal cord ependymomas: perioperative neurological functions and surgical outcomes.
Yang C, Sun J, Xie J, Ma C, Liu B, Wang T, Chen X, Wu J, Wu H, Zheng M, Chang Q, Yang J
Neurosurg Rev 2022 Feb;45(1):553-560. Epub 2021 May 14 doi: 10.1007/s10143-021-01567-5. PMID: 33988802
MRI characteristics and resectability in spinal cord glioma.
Seaman SC, Bathla G, Park BJ, Woodroffe RW, Smith M, Menezes AH, Noeller J, Yamaguchi S, Hitchon PW
Clin Neurol Neurosurg 2021 Jan;200:106321. Epub 2020 Oct 19 doi: 10.1016/j.clineuro.2020.106321. PMID: 33268194
Intramedullary spinal cord ependymoma and astrocytoma: intraoperative frozen-section diagnosis, extent of resection, and outcomes.
Hongo H, Takai K, Komori T, Taniguchi M
J Neurosurg Spine 2018 Oct 19;30(1):133-139. doi: 10.3171/2018.7.SPINE18230. PMID: 30485241
Clinical outcomes of radiotherapy for spinal cord ependymoma with adverse prognostic features: a single-center study.
Byun HK, Yi S, Yoon HI, Kim SH, Cho J, Suh CO
J Neurooncol 2018 Dec;140(3):649-657. Epub 2018 Sep 10 doi: 10.1007/s11060-018-2995-1. PMID: 30203166
Natural history of intramedullary spinal cord ependymoma in patients preferring nonoperative treatment.
Behmanesh B, Gessler F, Dützmann S, Dubinski D, Imoehl L, Seifert V, Setzer M, Marquardt G
J Neurooncol 2017 Oct;135(1):93-98. Epub 2017 Jun 30 doi: 10.1007/s11060-017-2552-3. PMID: 28667596

Recent systematic reviews

Bevacizumab as a surgery-sparing agent for spinal ependymoma in patients with neurofibromatosis type II: Systematic review and case.
Snyder MH, Ampie L, DiDomenico JD, Asthagiri AR
J Clin Neurosci 2021 Apr;86:79-84. Epub 2021 Feb 1 doi: 10.1016/j.jocn.2021.01.010. PMID: 33775351

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    Curated

    • NCCN, 2023
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Central Nervous System Cancers, 2023

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    Reviews

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