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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
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

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
Factors Affecting Long-Term Surgical Outcomes of Spinal Extramedullary Ependymomas: A Retrospective Study.
Abdallah A, Baloğlu G, Güler Abdallah B, Gündağ Papaker M
World Neurosurg 2022 Nov;167:e877-e890. Epub 2022 Aug 29 doi: 10.1016/j.wneu.2022.08.110. PMID: 36049726
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
Surgical Treatment of Spinal Ependymomas: Experience in 49 Patients.
Wild F, Hartmann C, Heissler HE, Hong B, Krauss JK, Nakamura M
World Neurosurg 2018 Mar;111:e703-e709. Epub 2018 Jan 5 doi: 10.1016/j.wneu.2017.12.159. PMID: 29309981
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

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
Giant myxopapillary ependymoma of the lumbosacral spine.
El Kacemi I, Miloudi G
Pan Afr Med J 2021;39:164. Epub 2021 Jul 1 doi: 10.11604/pamj.2021.39.164.29765. PMID: 34539960Free PMC Article
Ependymoma: a heterogeneous tumor of uncertain origin and limited therapeutic options.
Dorfer C, Tonn J, Rutka JT
Handb Clin Neurol 2016;134:417-31. doi: 10.1016/B978-0-12-802997-8.00025-6. PMID: 26948369
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
Thalidomide neuropathy in childhood.
Fleming FJ, Vytopil M, Chaitow J, Jones HR Jr, Darras BT, Ryan MM
Neuromuscul Disord 2005 Feb;15(2):172-6. Epub 2004 Nov 26 doi: 10.1016/j.nmd.2004.10.005. PMID: 15694139
Experience with tamoxifen/etoposide in the treatment of a child with myxopapillary ependymoma.
Madden JR, Fenton LZ, Weil M, Winston KR, Partington M, Foreman NK
Med Pediatr Oncol 2001 Jul;37(1):67-9. doi: 10.1002/mpo.1168. PMID: 11466729

Prognosis

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
Benign ependymoma with extensive intracranial and spinal cerebrospinal fluid dissemination: case report and literature review.
Zhu F, Ding J, Li Y, Mao D, He X, Chen W, Lou L, Ding Z
Br J Neurosurg 2019 Jun;33(3):290-293. Epub 2017 Jun 21 doi: 10.1080/02688697.2017.1340584. PMID: 28633540
Surgical Treatment of Spinal Ependymomas: Experience in 49 Patients.
Wild F, Hartmann C, Heissler HE, Hong B, Krauss JK, Nakamura M
World Neurosurg 2018 Mar;111:e703-e709. Epub 2018 Jan 5 doi: 10.1016/j.wneu.2017.12.159. PMID: 29309981
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
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
Spinal myxopapillary ependymomas: a retrospective clinical and immunohistochemical study.
Chen X, Li C, Che X, Chen H, Liu Z
Acta Neurochir (Wien) 2016 Jan;158(1):101-7. Epub 2015 Nov 17 doi: 10.1007/s00701-015-2637-8. PMID: 26577638
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

Resection of intradural spinal lesions with concomitant instrumented fusion in children: a systematic review and representative cases.
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
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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