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Adult spinal cord ependymoma

MedGen UID:
231359
•Concept ID:
C1332215
•
Neoplastic Process
Synonyms: Adult Spinal Cord Ependymoma; adult spinal cord ependymoma; Adult Spinal Cord Ependymoma without MYCN Amplification; Adult Spinal Ependymoma; spinal cord ependymoma of adults
 
Monarch Initiative: MONDO:0004361

Definition

An ependymoma of the spinal cord not associated with MYCN amplification and occurring in adults. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Adult spinal cord ependymoma

Professional guidelines

PubMed

Ependymoma: Evaluation and Management Updates.
Rudà R, Bruno F, Pellerino A, Soffietti R
Curr Oncol Rep 2022 Aug;24(8):985-993. Epub 2022 Apr 6 doi: 10.1007/s11912-022-01260-w. PMID: 35384591Free PMC Article
Intradural spinal tumors in adults-update on management and outcome.
Ottenhausen M, Ntoulias G, Bodhinayake I, Ruppert FH, Schreiber S, Förschler A, Boockvar JA, Jödicke A
Neurosurg Rev 2019 Jun;42(2):371-388. Epub 2018 Feb 17 doi: 10.1007/s10143-018-0957-x. PMID: 29455369
Clinical management of intramedullary spinal ependymomas in adults.
Lee J, Parsa AT, Ames CP, McCormick PC
Neurosurg Clin N Am 2006 Jan;17(1):21-7. doi: 10.1016/j.nec.2005.11.001. PMID: 16448904

Recent clinical studies

Etiology

Benign Glioma.
Wu PB, Filley AC, Miller ML, Bruce JN
Adv Exp Med Biol 2023;1405:31-71. doi: 10.1007/978-3-031-23705-8_2. PMID: 37452934
Ependymoma: Evaluation and Management Updates.
Rudà R, Bruno F, Pellerino A, Soffietti R
Curr Oncol Rep 2022 Aug;24(8):985-993. Epub 2022 Apr 6 doi: 10.1007/s11912-022-01260-w. PMID: 35384591Free PMC Article
Diagnostic, therapeutic, and prognostic implications of the 2021 World Health Organization classification of tumors of the central nervous system.
Gritsch S, Batchelor TT, Gonzalez Castro LN
Cancer 2022 Jan 1;128(1):47-58. Epub 2021 Oct 11 doi: 10.1002/cncr.33918. PMID: 34633681
Challenges in diagnosis and management of adult spinal cord gliomas.
Campello C, Tabouret E, Chinot O
Rev Neurol (Paris) 2021 May;177(5):515-523. Epub 2021 Apr 23 doi: 10.1016/j.neurol.2021.02.384. PMID: 33896651
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

Diagnosis

Ependymoma: Evaluation and Management Updates.
Rudà R, Bruno F, Pellerino A, Soffietti R
Curr Oncol Rep 2022 Aug;24(8):985-993. Epub 2022 Apr 6 doi: 10.1007/s11912-022-01260-w. PMID: 35384591Free PMC Article
Intradural spinal tumors in adults-update on management and outcome.
Ottenhausen M, Ntoulias G, Bodhinayake I, Ruppert FH, Schreiber S, Förschler A, Boockvar JA, Jödicke A
Neurosurg Rev 2019 Jun;42(2):371-388. Epub 2018 Feb 17 doi: 10.1007/s10143-018-0957-x. PMID: 29455369
Cystic extramedullary ependymoma.
Graça J, Gültasli N, D'Haene N, Brotchi J, Salmon I, Balériaux D
AJNR Am J Neuroradiol 2006 Apr;27(4):818-21. PMID: 16611771Free PMC Article
Ependymomas.
Chamberlain MC
Curr Neurol Neurosci Rep 2003 May;3(3):193-9. doi: 10.1007/s11910-003-0078-x. PMID: 12691623
Ultrastructure of ependymoma.
Sara A, Bruner JM, Mackay B
Ultrastruct Pathol 1994 Jan-Apr;18(1-2):33-42. doi: 10.3109/01913129409016272. PMID: 8191645

Therapy

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
CNS Tumors in Neurofibromatosis.
Campian J, Gutmann DH
J Clin Oncol 2017 Jul 20;35(21):2378-2385. Epub 2017 Jun 22 doi: 10.1200/JCO.2016.71.7199. PMID: 28640700Free PMC Article
Spinal cord anaplastic ependymoma induced by radiation treatment of breast cancer.
Ishihara M, Iwatsuki K, Chatani M, Yamamoto K, Yoshimine T
J Neurosurg Sci 2017 Aug;61(4):454-455. doi: 10.23736/S0390-5616.16.03399-3. PMID: 28555490
Ependymomas.
Chamberlain MC
Curr Neurol Neurosci Rep 2003 May;3(3):193-9. doi: 10.1007/s11910-003-0078-x. PMID: 12691623
Etoposide for recurrent spinal cord ependymoma.
Chamberlain MC
Neurology 2002 Apr 23;58(8):1310-1. doi: 10.1212/wnl.58.8.1310. PMID: 11971113

Prognosis

Benign Glioma.
Wu PB, Filley AC, Miller ML, Bruce JN
Adv Exp Med Biol 2023;1405:31-71. doi: 10.1007/978-3-031-23705-8_2. PMID: 37452934
Ependymoma: Evaluation and Management Updates.
Rudà R, Bruno F, Pellerino A, Soffietti R
Curr Oncol Rep 2022 Aug;24(8):985-993. Epub 2022 Apr 6 doi: 10.1007/s11912-022-01260-w. PMID: 35384591Free PMC Article
Diagnostic, therapeutic, and prognostic implications of the 2021 World Health Organization classification of tumors of the central nervous system.
Gritsch S, Batchelor TT, Gonzalez Castro LN
Cancer 2022 Jan 1;128(1):47-58. Epub 2021 Oct 11 doi: 10.1002/cncr.33918. PMID: 34633681
Pediatric Spinal Cord Diseases.
Lu VM, Niazi TN
Pediatr Rev 2021 Sep;42(9):486-499. doi: 10.1542/pir.2020-000661. PMID: 34470868
Ependymomas.
Chamberlain MC
Curr Neurol Neurosci Rep 2003 May;3(3):193-9. doi: 10.1007/s11910-003-0078-x. PMID: 12691623

Clinical prediction guides

Distinct relapse pattern across molecular ependymoma types.
Obrecht-Sturm D, Schoof M, Eckhardt A, Mynarek M, Gilbert MR, Aldape K, Armstrong TS, Ramaswamy V, Bockmayr M, von Hoff K, Fleischhack G, Adolph JE, Tippelt S, Pfister SM, Pajtler K, Sturm D, Drexler R, Ricklefs FL, Stepien N, Gojo J, Pietsch T, Warmuth-Metz M, Kortmann R, Timmermann B, Haberler C, Rutkowski S, Schüller U
Neuro Oncol 2025 Jan 12;27(1):267-276. doi: 10.1093/neuonc/noae166. PMID: 39171767Free PMC Article
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
Rhabdoid type intramedullary meningioma. A case report and review of the literature.
Riqué Dormido J, Gómez Cárdenas E, Marín Láut FM, Millan Ortega I
Neurocirugia (Astur : Engl Ed) 2019 Jul-Aug;30(4):202-205. Epub 2018 Sep 12 doi: 10.1016/j.neucir.2018.08.001. PMID: 30219414
Adult primary intradural spinal cord tumors: a review.
Chamberlain MC, Tredway TL
Curr Neurol Neurosci Rep 2011 Jun;11(3):320-8. doi: 10.1007/s11910-011-0190-2. PMID: 21327734
Ependymomas in adults.
Gilbert MR, Ruda R, Soffietti R
Curr Neurol Neurosci Rep 2010 May;10(3):240-7. doi: 10.1007/s11910-010-0109-3. PMID: 20425040

Recent systematic reviews

Clinical Characteristics, Management, and Outcomes of Intramedullary Spinal Cord Ependymomas in Adults: A Systematic Review.
Farooqi S, Tebha SS, Qamar MA, Singh S, Alfawares Y, Ramanathan V, Haider AS, Ferini G, Sharma M, Umana GE, Aoun SG, Palmisciano P
World Neurosurg 2023 May;173:237-250.e8. Epub 2023 Feb 28 doi: 10.1016/j.wneu.2023.02.098. PMID: 36858296
Novel Clinical Insights into Spinal Hemangioblastoma in Adults: A Systematic Review.
Jankovic D, Hanissian A, Rotim K, Splavski B, Arnautovic KI
World Neurosurg 2022 Feb;158:1-10. Epub 2021 Oct 21 doi: 10.1016/j.wneu.2021.10.105. PMID: 34687932
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
A systematic review of outcome in intramedullary ependymoma and astrocytoma.
Hamilton KR, Lee SS, Urquhart JC, Jonker BP
J Clin Neurosci 2019 May;63:168-175. Epub 2019 Mar 2 doi: 10.1016/j.jocn.2019.02.001. PMID: 30833131
Spinal cord ependymomas and the appearance of other de novo tumors: a systematic review.
Fotakopoulos G, Vagkopoulos K, Gatos C, Kotlia P, Brotis A
J Med Case Rep 2014 Dec 18;8:438. doi: 10.1186/1752-1947-8-438. PMID: 25519213Free PMC Article

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