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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.
 
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

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
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
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

Recent clinical studies

Etiology

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
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
Abdallah A, Emel E, Gündüz HB, Sofuoğlu ÖE, Asiltürk M, Abdallah BG
World Neurosurg 2020 Apr;136:e245-e261. Epub 2019 Dec 30 doi: 10.1016/j.wneu.2019.12.128. PMID: 31899399
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
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

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
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
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
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
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

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
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
Kurup AN, Woodrum DA, Morris JM, Atwell TD, Schmit GD, Welch TJ, Yaszemski MJ, Callstrom MR
J Vasc Interv Radiol 2012 Aug;23(8):1070-5. doi: 10.1016/j.jvir.2012.05.043. PMID: 22840806
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
Sakai Y, Matsuyama Y, Katayama Y, Imagama S, Ito Z, Wakao N, Kanemura T, Yoshida G, Sato K, Ando T, Nakamura H, Kato F, Yukawa Y, Ito K, Ishiguro N
Spine (Phila Pa 1976) 2009 Jul 1;34(15):1619-24. doi: 10.1097/BRS.0b013e3181a983d8. PMID: 19564773

Prognosis

Almatrafi FR, Aseeri AM, Alqahtani MF, Al Mulla L, Al-Jubran S, AlOmair MA, Alsalmi S, Al-Anazi R
Med Arch 2023 Apr;77(2):150-154. doi: 10.5455/medarh.2023.77.150-154. PMID: 37260799Free PMC Article
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
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
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
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

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
Gitto L, Serinelli S, Galbraith K, Williams M, Mirchia K, Galgano MA, Krishnamurthy S, de la Roza G, Viapiano MS, Walker JM, Jour G, Serrano J, DeLorenzo M, Snuderl M, Richardson TE
J Neuropathol Exp Neurol 2020 Oct 1;79(10):1044-1053. doi: 10.1093/jnen/nlaa077. PMID: 32743660
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
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
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

Prather KY, Baier MP, Coulibaly NJ, Stephens ME, Chen S, Omini ME, Jea A
J Neurosurg Pediatr 2024 Jul 1;34(1):99-110. Epub 2024 Apr 5 doi: 10.3171/2024.1.PEDS23444. PMID: 38579345
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
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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