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Adult Myxopapillary Ependymoma

MedGen UID:
854377
•Concept ID:
C3887518
•
Neoplastic Process

Definition

A myxopapillary ependymoma that occurs during adulthood. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAdult Myxopapillary 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
EANO guidelines for the diagnosis and treatment of ependymal tumors.
Rudà R, Reifenberger G, Frappaz D, Pfister SM, Laprie A, Santarius T, Roth P, Tonn JC, Soffietti R, Weller M, Moyal EC
Neuro Oncol 2018 Mar 27;20(4):445-456. doi: 10.1093/neuonc/nox166. PMID: 29194500Free PMC Article

Recent clinical studies

Etiology

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
Conformal Radiation Therapy for Pediatric Ependymoma, Chemotherapy for Incompletely Resected Ependymoma, and Observation for Completely Resected, Supratentorial Ependymoma.
Merchant TE, Bendel AE, Sabin ND, Burger PC, Shaw DW, Chang E, Wu S, Zhou T, Eisenstat DD, Foreman NK, Fuller CE, Anderson ET, Hukin J, Lau CC, Pollack IF, Laningham FH, Lustig RH, Armstrong FD, Handler MH, Williams-Hughes C, Kessel S, Kocak M, Ellison DW, Ramaswamy V
J Clin Oncol 2019 Apr 20;37(12):974-983. Epub 2019 Feb 27 doi: 10.1200/JCO.18.01765. PMID: 30811284Free PMC Article
Ependymoma.
Gerstner ER, Pajtler KW
Semin Neurol 2018 Feb;38(1):104-111. Epub 2018 Mar 16 doi: 10.1055/s-0038-1636503. PMID: 29548057
Adjuvant treatments for ependymomas.
Sartor EA, Wen PY
J Neurosurg Sci 2018 Feb;62(1):71-77. Epub 2017 Sep 22 doi: 10.23736/S0390-5616.17.04211-4. PMID: 28945055
Molecular Classification of Ependymal Tumors across All CNS Compartments, Histopathological Grades, and Age Groups.
Pajtler KW, Witt H, Sill M, Jones DT, Hovestadt V, Kratochwil F, Wani K, Tatevossian R, Punchihewa C, Johann P, Reimand J, Warnatz HJ, Ryzhova M, Mack S, Ramaswamy V, Capper D, Schweizer L, Sieber L, Wittmann A, Huang Z, van Sluis P, Volckmann R, Koster J, Versteeg R, Fults D, Toledano H, Avigad S, Hoffman LM, Donson AM, Foreman N, Hewer E, Zitterbart K, Gilbert M, Armstrong TS, Gupta N, Allen JC, Karajannis MA, Zagzag D, Hasselblatt M, Kulozik AE, Witt O, Collins VP, von Hoff K, Rutkowski S, Pietsch T, Bader G, Yaspo ML, von Deimling A, Lichter P, Taylor MD, Gilbertson R, Ellison DW, Aldape K, Korshunov A, Kool M, Pfister SM
Cancer Cell 2015 May 11;27(5):728-43. doi: 10.1016/j.ccell.2015.04.002. PMID: 25965575Free 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
Orbital Ependymoma.
Tsimchi IM, Malik AI, Takashima M, Lee AG
Ophthalmic Plast Reconstr Surg 2022 Mar-Apr 01;38(2):e51-e54. doi: 10.1097/IOP.0000000000002095. PMID: 35030150
Conformal Radiation Therapy for Pediatric Ependymoma, Chemotherapy for Incompletely Resected Ependymoma, and Observation for Completely Resected, Supratentorial Ependymoma.
Merchant TE, Bendel AE, Sabin ND, Burger PC, Shaw DW, Chang E, Wu S, Zhou T, Eisenstat DD, Foreman NK, Fuller CE, Anderson ET, Hukin J, Lau CC, Pollack IF, Laningham FH, Lustig RH, Armstrong FD, Handler MH, Williams-Hughes C, Kessel S, Kocak M, Ellison DW, Ramaswamy V
J Clin Oncol 2019 Apr 20;37(12):974-983. Epub 2019 Feb 27 doi: 10.1200/JCO.18.01765. PMID: 30811284Free PMC Article
Ependymoma.
Gerstner ER, Pajtler KW
Semin Neurol 2018 Feb;38(1):104-111. Epub 2018 Mar 16 doi: 10.1055/s-0038-1636503. PMID: 29548057
EANO guidelines for the diagnosis and treatment of ependymal tumors.
Rudà R, Reifenberger G, Frappaz D, Pfister SM, Laprie A, Santarius T, Roth P, Tonn JC, Soffietti R, Weller M, Moyal EC
Neuro Oncol 2018 Mar 27;20(4):445-456. doi: 10.1093/neuonc/nox166. PMID: 29194500Free PMC Article

Therapy

Major Features of the 2021 WHO Classification of CNS Tumors.
Smith HL, Wadhwani N, Horbinski C
Neurotherapeutics 2022 Oct;19(6):1691-1704. Epub 2022 May 16 doi: 10.1007/s13311-022-01249-0. PMID: 35578106Free PMC Article
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
Conformal Radiation Therapy for Pediatric Ependymoma, Chemotherapy for Incompletely Resected Ependymoma, and Observation for Completely Resected, Supratentorial Ependymoma.
Merchant TE, Bendel AE, Sabin ND, Burger PC, Shaw DW, Chang E, Wu S, Zhou T, Eisenstat DD, Foreman NK, Fuller CE, Anderson ET, Hukin J, Lau CC, Pollack IF, Laningham FH, Lustig RH, Armstrong FD, Handler MH, Williams-Hughes C, Kessel S, Kocak M, Ellison DW, Ramaswamy V
J Clin Oncol 2019 Apr 20;37(12):974-983. Epub 2019 Feb 27 doi: 10.1200/JCO.18.01765. PMID: 30811284Free PMC Article
Cerebellar tumors.
Guerreiro Stucklin AS, Grotzer MA
Handb Clin Neurol 2018;155:289-299. doi: 10.1016/B978-0-444-64189-2.00019-6. PMID: 29891066
Adjuvant treatments for ependymomas.
Sartor EA, Wen PY
J Neurosurg Sci 2018 Feb;62(1):71-77. Epub 2017 Sep 22 doi: 10.23736/S0390-5616.17.04211-4. PMID: 28945055

Prognosis

Myxopapillary Ependymoma-a Case Report of Rare Multicentric Subtype and Literature Review.
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
Adult intracranial ependymoma-relevance of DNA methylation profiling for diagnosis, prognosis, and treatment.
Träger M, Schweizer L, Pérez E, Schmid S, Hain EG, Dittmayer C, Onken J, Fukuoka K, Ichimura K, Schüller U, Dührsen L, Müther M, Paulus W, Thomas C, Gutt-Will M, Schucht P, Maragkou T, Schittenhelm J, Eckert F, Niyazi M, Fleischmann DF, Dorostkar MM, Feyer P, May SA, Moskopp D, Badakhshi H, Radke C, Walter J, Ehret F, Capper D, Kaul D
Neuro Oncol 2023 Jul 6;25(7):1286-1298. doi: 10.1093/neuonc/noad030. PMID: 36734226Free 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
EANO guidelines for the diagnosis and treatment of ependymal tumors.
Rudà R, Reifenberger G, Frappaz D, Pfister SM, Laprie A, Santarius T, Roth P, Tonn JC, Soffietti R, Weller M, Moyal EC
Neuro Oncol 2018 Mar 27;20(4):445-456. doi: 10.1093/neuonc/nox166. PMID: 29194500Free PMC Article
Molecular Classification of Ependymal Tumors across All CNS Compartments, Histopathological Grades, and Age Groups.
Pajtler KW, Witt H, Sill M, Jones DT, Hovestadt V, Kratochwil F, Wani K, Tatevossian R, Punchihewa C, Johann P, Reimand J, Warnatz HJ, Ryzhova M, Mack S, Ramaswamy V, Capper D, Schweizer L, Sieber L, Wittmann A, Huang Z, van Sluis P, Volckmann R, Koster J, Versteeg R, Fults D, Toledano H, Avigad S, Hoffman LM, Donson AM, Foreman N, Hewer E, Zitterbart K, Gilbert M, Armstrong TS, Gupta N, Allen JC, Karajannis MA, Zagzag D, Hasselblatt M, Kulozik AE, Witt O, Collins VP, von Hoff K, Rutkowski S, Pietsch T, Bader G, Yaspo ML, von Deimling A, Lichter P, Taylor MD, Gilbertson R, Ellison DW, Aldape K, Korshunov A, Kool M, Pfister SM
Cancer Cell 2015 May 11;27(5):728-43. doi: 10.1016/j.ccell.2015.04.002. PMID: 25965575Free PMC Article

Clinical prediction guides

Adult intracranial ependymoma-relevance of DNA methylation profiling for diagnosis, prognosis, and treatment.
Träger M, Schweizer L, Pérez E, Schmid S, Hain EG, Dittmayer C, Onken J, Fukuoka K, Ichimura K, Schüller U, Dührsen L, Müther M, Paulus W, Thomas C, Gutt-Will M, Schucht P, Maragkou T, Schittenhelm J, Eckert F, Niyazi M, Fleischmann DF, Dorostkar MM, Feyer P, May SA, Moskopp D, Badakhshi H, Radke C, Walter J, Ehret F, Capper D, Kaul D
Neuro Oncol 2023 Jul 6;25(7):1286-1298. doi: 10.1093/neuonc/noad030. PMID: 36734226Free PMC Article
Conformal Radiation Therapy for Pediatric Ependymoma, Chemotherapy for Incompletely Resected Ependymoma, and Observation for Completely Resected, Supratentorial Ependymoma.
Merchant TE, Bendel AE, Sabin ND, Burger PC, Shaw DW, Chang E, Wu S, Zhou T, Eisenstat DD, Foreman NK, Fuller CE, Anderson ET, Hukin J, Lau CC, Pollack IF, Laningham FH, Lustig RH, Armstrong FD, Handler MH, Williams-Hughes C, Kessel S, Kocak M, Ellison DW, Ramaswamy V
J Clin Oncol 2019 Apr 20;37(12):974-983. Epub 2019 Feb 27 doi: 10.1200/JCO.18.01765. PMID: 30811284Free PMC Article
Molecular Classification of Ependymal Tumors across All CNS Compartments, Histopathological Grades, and Age Groups.
Pajtler KW, Witt H, Sill M, Jones DT, Hovestadt V, Kratochwil F, Wani K, Tatevossian R, Punchihewa C, Johann P, Reimand J, Warnatz HJ, Ryzhova M, Mack S, Ramaswamy V, Capper D, Schweizer L, Sieber L, Wittmann A, Huang Z, van Sluis P, Volckmann R, Koster J, Versteeg R, Fults D, Toledano H, Avigad S, Hoffman LM, Donson AM, Foreman N, Hewer E, Zitterbart K, Gilbert M, Armstrong TS, Gupta N, Allen JC, Karajannis MA, Zagzag D, Hasselblatt M, Kulozik AE, Witt O, Collins VP, von Hoff K, Rutkowski S, Pietsch T, Bader G, Yaspo ML, von Deimling A, Lichter P, Taylor MD, Gilbertson R, Ellison DW, Aldape K, Korshunov A, Kool M, Pfister SM
Cancer Cell 2015 May 11;27(5):728-43. doi: 10.1016/j.ccell.2015.04.002. PMID: 25965575Free PMC Article
Molecular genetics of ependymoma.
Yao Y, Mack SC, Taylor MD
Chin J Cancer 2011 Oct;30(10):669-81. doi: 10.5732/cjc.011.10129. PMID: 21959044Free PMC Article
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

The effectiveness and safety of proton beam radiation therapy in children and young adults with Central Nervous System (CNS) tumours: a systematic review.
Wilson JS, Main C, Thorp N, Taylor RE, Majothi S, Kearns PR, English M, Dandapani M, Phillips R, Wheatley K, Pizer B
J Neurooncol 2024 Mar;167(1):1-34. Epub 2024 Jan 31 doi: 10.1007/s11060-023-04510-4. PMID: 38294638Free PMC Article
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
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
Proton Therapy in Children: A Systematic Review of Clinical Effectiveness in 15 Pediatric Cancers.
Leroy R, Benahmed N, Hulstaert F, Van Damme N, De Ruysscher D
Int J Radiat Oncol Biol Phys 2016 May 1;95(1):267-278. Epub 2015 Oct 17 doi: 10.1016/j.ijrobp.2015.10.025. PMID: 27084646
Tumor control after surgery for spinal myxopapillary ependymomas: distinct outcomes in adults versus children: a systematic review.
Feldman WB, Clark AJ, Safaee M, Ames CP, Parsa AT
J Neurosurg Spine 2013 Oct;19(4):471-6. Epub 2013 Aug 23 doi: 10.3171/2013.6.SPINE12927. PMID: 23971762

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