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Myxopapillary ependymoma

MedGen UID:
104717
•Concept ID:
C0205769
•
Neoplastic Process
Synonyms: Ependymoma, Myxopapillary; Ependymomas, Myxopapillary; Myxopapillary Ependymoma; Myxopapillary Ependymomas
SNOMED CT: Myxopapillary ependymoma (1623000)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0016699
Orphanet: ORPHA251643

Definition

Myxopapillary ependymoma (MEPN) describes a slow growing ependymoma located almost exclusively in the conus medullaris-cauda equina-filum terminale region of the spinal cord, presenting in all age groups, and manifesting with variable symptoms such as neck pain, vomiting and unsteady gait and metastasis. It has a more aggressive disease course and is seen in the pediatric population. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMyxopapillary ependymoma
Follow this link to review classifications for Myxopapillary ependymoma in Orphanet.

Professional guidelines

PubMed

Clinical management and prognosis of spinal myxopapillary ependymoma: a single-institution cohort of 72 patients.
Zhang YW, Wang B, An SY, Liu WH, Wang C, Yan H, Xu YL, Wang YZ, Jia WQ
Eur Spine J 2023 Jul;32(7):2459-2467. Epub 2023 Apr 7 doi: 10.1007/s00586-023-07690-9. PMID: 37027035
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
Metastases of spinal myxopapillary ependymoma: unique characteristics and clinical management.
Kraetzig T, McLaughlin L, Bilsky MH, Laufer I
J Neurosurg Spine 2018 Feb;28(2):201-208. Epub 2017 Dec 8 doi: 10.3171/2017.5.SPINE161164. PMID: 29219779

Recent clinical studies

Etiology

Ependymomas.
Bertero L, Ricci AA, Tampieri C, Cassoni P, Modena P
Pathologica 2022 Dec;114(6):436-446. doi: 10.32074/1591-951X-817. PMID: 36534422Free PMC Article
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
cIMPACT-NOW update 7: advancing the molecular classification of ependymal tumors.
Ellison DW, Aldape KD, Capper D, Fouladi M, Gilbert MR, Gilbertson RJ, Hawkins C, Merchant TE, Pajtler K, Venneti S, Louis DN
Brain Pathol 2020 Sep;30(5):863-866. Epub 2020 Jun 23 doi: 10.1111/bpa.12866. PMID: 32502305Free PMC Article
Characterization of gliomas: from morphology to molecules.
Ferris SP, Hofmann JW, Solomon DA, Perry A
Virchows Arch 2017 Aug;471(2):257-269. Epub 2017 Jul 4 doi: 10.1007/s00428-017-2181-4. PMID: 28674742
Ependymoma.
Reni M, Gatta G, Mazza E, Vecht C
Crit Rev Oncol Hematol 2007 Jul;63(1):81-9. Epub 2007 May 4 doi: 10.1016/j.critrevonc.2007.03.004. PMID: 17482475

Diagnosis

Intradural Extramedullary Tumors and Associated Syndromes.
Puac-Polanco P, Guarnizo A, Cruz JP, Rodriguez FR, Torres CH
Neuroimaging Clin N Am 2023 Aug;33(3):407-422. Epub 2023 Apr 25 doi: 10.1016/j.nic.2023.03.002. PMID: 37356859
Ependymomas.
Bertero L, Ricci AA, Tampieri C, Cassoni P, Modena P
Pathologica 2022 Dec;114(6):436-446. doi: 10.32074/1591-951X-817. PMID: 36534422Free PMC Article
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
Characterization of gliomas: from morphology to molecules.
Ferris SP, Hofmann JW, Solomon DA, Perry A
Virchows Arch 2017 Aug;471(2):257-269. Epub 2017 Jul 4 doi: 10.1007/s00428-017-2181-4. PMID: 28674742
Ependymoma.
Reni M, Gatta G, Mazza E, Vecht C
Crit Rev Oncol Hematol 2007 Jul;63(1):81-9. Epub 2007 May 4 doi: 10.1016/j.critrevonc.2007.03.004. PMID: 17482475

Therapy

Metastatic myxopapillary ependymoma treated with immunotherapy achieving durable response.
Tapia Rico G, Townsend A, Price T, Patterson K
BMJ Case Rep 2020 Dec 17;13(12) doi: 10.1136/bcr-2020-236242. PMID: 33334744Free PMC Article
Remarkable efficacy of temozolomide for relapsed spinal myxopapillary ependymoma with multiple recurrence and cerebrospinal dissemination: a case report and literature review.
Fujiwara Y, Manabe H, Izumi B, Shima T, Adachi N
Eur Spine J 2018 Jul;27(Suppl 3):421-425. Epub 2017 Dec 21 doi: 10.1007/s00586-017-5413-z. PMID: 29270703
You're the Flight Surgeon.
Aerosp Med Hum Perform 2017 Oct 1;88(10):970-973. doi: 10.3357/AMHP.4835.2017. PMID: 28923150
Outcome predictors in the management of spinal myxopapillary ependymoma: an integrative survival analysis.
Kukreja S, Ambekar S, Sharma M, Sin AH, Nanda A
World Neurosurg 2015 May;83(5):852-9. Epub 2014 Aug 7 doi: 10.1016/j.wneu.2014.08.006. PMID: 25108296
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

Prognosis

Clinical management and prognosis of spinal myxopapillary ependymoma: a single-institution cohort of 72 patients.
Zhang YW, Wang B, An SY, Liu WH, Wang C, Yan H, Xu YL, Wang YZ, Jia WQ
Eur Spine J 2023 Jul;32(7):2459-2467. Epub 2023 Apr 7 doi: 10.1007/s00586-023-07690-9. PMID: 37027035
Ependymomas.
Bertero L, Ricci AA, Tampieri C, Cassoni P, Modena P
Pathologica 2022 Dec;114(6):436-446. doi: 10.32074/1591-951X-817. PMID: 36534422Free PMC Article
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
Characterization of gliomas: from morphology to molecules.
Ferris SP, Hofmann JW, Solomon DA, Perry A
Virchows Arch 2017 Aug;471(2):257-269. Epub 2017 Jul 4 doi: 10.1007/s00428-017-2181-4. PMID: 28674742
Ependymoma.
Reni M, Gatta G, Mazza E, Vecht C
Crit Rev Oncol Hematol 2007 Jul;63(1):81-9. Epub 2007 May 4 doi: 10.1016/j.critrevonc.2007.03.004. PMID: 17482475

Clinical prediction guides

Clinical management and prognosis of spinal myxopapillary ependymoma: a single-institution cohort of 72 patients.
Zhang YW, Wang B, An SY, Liu WH, Wang C, Yan H, Xu YL, Wang YZ, Jia WQ
Eur Spine J 2023 Jul;32(7):2459-2467. Epub 2023 Apr 7 doi: 10.1007/s00586-023-07690-9. PMID: 37027035
Clinical characteristics and long-term surgical outcome of spinal myxopapillary ependymoma: a French cohort of 101 patients.
Montero AS, Tran S, Amelot A, Berriat F, Lot G, Gaillard S, Villa C, Polivka M, Adam C, Idbaih A, Feuvret L, Carpentier A, Parker F, Bielle F, Mathon B
J Neurooncol 2021 May;152(3):491-499. Epub 2021 Feb 23 doi: 10.1007/s11060-021-03717-7. PMID: 33624261
Metastases of spinal myxopapillary ependymoma: unique characteristics and clinical management.
Kraetzig T, McLaughlin L, Bilsky MH, Laufer I
J Neurosurg Spine 2018 Feb;28(2):201-208. Epub 2017 Dec 8 doi: 10.3171/2017.5.SPINE161164. PMID: 29219779
Characterization of gliomas: from morphology to molecules.
Ferris SP, Hofmann JW, Solomon DA, Perry A
Virchows Arch 2017 Aug;471(2):257-269. Epub 2017 Jul 4 doi: 10.1007/s00428-017-2181-4. PMID: 28674742
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

Primary extraskeletal intradural Ewing sarcoma with acute hemorrhage: a case report and review of the literature.
Salama H, Abu-Hilal LH, Idkedek M, Kharousha A, Abulihya M, Nimer H
J Med Case Rep 2024 Mar 9;18(1):144. doi: 10.1186/s13256-024-04384-8. PMID: 38459600Free PMC Article
Spinal Myxopapillary Ependymoma: The Sapienza University Experience and Comprehensive Literature Review Concerning the Clinical Course of 1602 Patients.
Pesce A, Palmieri M, Armocida D, Frati A, Miscusi M, Raco A
World Neurosurg 2019 Sep;129:245-253. Epub 2019 May 29 doi: 10.1016/j.wneu.2019.05.206. PMID: 31152881

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