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Anaplastic ependymoma

MedGen UID:
128891
•Concept ID:
C0280788
•
Neoplastic Process
Synonym: Malignant ependymoma
SNOMED CT: Ependymoma, anaplastic (21589007); Malignant ependymoma (1156903009); Malignant epithelial ependymoma (1156903009)
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:0016700
Orphanet: ORPHA251646

Definition

A rare, malignant type of ependymoma that most often arises in the supratentorial region of the brain of children and young adults and that manifests with variable symptoms including headaches, nausea, vision impairment, memory loss and difficulty walking. [from ORDO]

Term Hierarchy

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

Professional guidelines

PubMed

Cancer treatment in disabled children.
Meazza C, Schiavello E, Biassoni V, Podda M, Barteselli C, Barretta F, Gattuso G, Terenziani M, Ferrari A, Spreafico F, Luksch R, Casanova M, Chiaravalli S, Puma N, Bergamaschi L, Massimino M
Eur J Pediatr 2020 Sep;179(9):1353-1360. Epub 2020 Mar 5 doi: 10.1007/s00431-020-03607-6. PMID: 32140854
DNA methylation-based classification of ependymomas in adulthood: implications for diagnosis and treatment.
Witt H, Gramatzki D, Hentschel B, Pajtler KW, Felsberg J, Schackert G, Löffler M, Capper D, Sahm F, Sill M, von Deimling A, Kool M, Herrlinger U, Westphal M, Pietsch T, Reifenberger G, Pfister SM, Tonn JC, Weller M; German Glioma Network
Neuro Oncol 2018 Nov 12;20(12):1616-1624. doi: 10.1093/neuonc/noy118. PMID: 30053291Free 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

Recent clinical studies

Etiology

Combined cytotoxic and immune-stimulatory gene therapy for primary adult high-grade glioma: a phase 1, first-in-human trial.
Umemura Y, Orringer D, Junck L, Varela ML, West MEJ, Faisal SM, Comba A, Heth J, Sagher O, Leung D, Mammoser A, Hervey-Jumper S, Zamler D, Yadav VN, Dunn P, Al-Holou W, Hollon T, Kim MM, Wahl DR, Camelo-Piragua S, Lieberman AP, Venneti S, McKeever P, Lawrence T, Kurokawa R, Sagher K, Altshuler D, Zhao L, Muraszko K, Castro MG, Lowenstein PR
Lancet Oncol 2023 Sep;24(9):1042-1052. doi: 10.1016/S1470-2045(23)00347-9. PMID: 37657463
Adult Posterior Fossa Anaplastic Ependymoma, Case Series and Literature Review.
Hsu HI, Hsu SS, Chung WY, Yip CM, Liu SH, Liao WC
World Neurosurg 2022 Feb;158:205-209. Epub 2021 Nov 26 doi: 10.1016/j.wneu.2021.11.074. PMID: 34839047
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
Epidemiology, molecular classification and WHO grading of ependymoma.
Hübner JM, Kool M, Pfister SM, Pajtler KW
J Neurosurg Sci 2018 Feb;62(1):46-50. Epub 2017 Sep 8 doi: 10.23736/S0390-5616.17.04152-2. PMID: 28895660
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

Recombinant polio-rhinovirus immunotherapy for recurrent paediatric high-grade glioma: a phase 1b trial.
Thompson EM, Landi D, Brown MC, Friedman HS, McLendon R, Herndon JE 2nd, Buckley E, Bolognesi DP, Lipp E, Schroeder K, Becher OJ, Friedman AH, McKay Z, Walter A, Threatt S, Jaggers D, Desjardins A, Gromeier M, Bigner DD, Ashley DM
Lancet Child Adolesc Health 2023 Jul;7(7):471-478. Epub 2023 Mar 30 doi: 10.1016/S2352-4642(23)00031-7. PMID: 37004712Free PMC Article
Adult Posterior Fossa Anaplastic Ependymoma, Case Series and Literature Review.
Hsu HI, Hsu SS, Chung WY, Yip CM, Liu SH, Liao WC
World Neurosurg 2022 Feb;158:205-209. Epub 2021 Nov 26 doi: 10.1016/j.wneu.2021.11.074. PMID: 34839047
The Spectrum of Underlying Diseases in Children with Torticollis.
Tumturk A, Gorkem SB, Ozmansur EN, Peduk Y, Arslan U, Gok V, Dogan H, Cetin BS, Sahin A, Gumus H, Guney A, Unal E, Kurtsoy A, Per H
Turk Neurosurg 2021;31(3):389-398. doi: 10.5137/1019-5149.JTN.31359-20.2. PMID: 33759163
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
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

Combined cytotoxic and immune-stimulatory gene therapy for primary adult high-grade glioma: a phase 1, first-in-human trial.
Umemura Y, Orringer D, Junck L, Varela ML, West MEJ, Faisal SM, Comba A, Heth J, Sagher O, Leung D, Mammoser A, Hervey-Jumper S, Zamler D, Yadav VN, Dunn P, Al-Holou W, Hollon T, Kim MM, Wahl DR, Camelo-Piragua S, Lieberman AP, Venneti S, McKeever P, Lawrence T, Kurokawa R, Sagher K, Altshuler D, Zhao L, Muraszko K, Castro MG, Lowenstein PR
Lancet Oncol 2023 Sep;24(9):1042-1052. doi: 10.1016/S1470-2045(23)00347-9. PMID: 37657463
Recombinant polio-rhinovirus immunotherapy for recurrent paediatric high-grade glioma: a phase 1b trial.
Thompson EM, Landi D, Brown MC, Friedman HS, McLendon R, Herndon JE 2nd, Buckley E, Bolognesi DP, Lipp E, Schroeder K, Becher OJ, Friedman AH, McKay Z, Walter A, Threatt S, Jaggers D, Desjardins A, Gromeier M, Bigner DD, Ashley DM
Lancet Child Adolesc Health 2023 Jul;7(7):471-478. Epub 2023 Mar 30 doi: 10.1016/S2352-4642(23)00031-7. PMID: 37004712Free PMC Article
Metronomic Cyclophosphamide With Cisplatin and Bevacizumab for Refractory Anaplastic Ependymoma: A Retrospective Study.
DE Laclos XC, Frappaz D, Ahle G, Bronnimann C, Catry-Thomas I, Joncour A, Ghiringhelli F, Vauleon E
Anticancer Res 2022 Aug;42(8):3863-3867. doi: 10.21873/anticanres.15878. PMID: 35896241
Nimotuzumab therapy in the treatment of pediatric central nervous system tumors: single-center experience.
Susam-Sen H, Varan A, Bajin İ, Göçmen R, Aydın B, Yalcin B, Kurucu N, Kutluk T, Bayhan T, Akyuz C
Naunyn Schmiedebergs Arch Pharmacol 2021 Aug;394(8):1769-1777. Epub 2021 Jun 21 doi: 10.1007/s00210-021-02109-y. PMID: 34151393
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

Prognosis

Single-cell RNA sequencing of anaplastic ependymoma and H3K27M-mutant diffuse midline glioma.
Zang D, Dong Z, Liu Y, Chen Q
BMC Neurol 2024 Feb 21;24(1):74. doi: 10.1186/s12883-024-03558-7. PMID: 38383423Free PMC Article
Combined cytotoxic and immune-stimulatory gene therapy for primary adult high-grade glioma: a phase 1, first-in-human trial.
Umemura Y, Orringer D, Junck L, Varela ML, West MEJ, Faisal SM, Comba A, Heth J, Sagher O, Leung D, Mammoser A, Hervey-Jumper S, Zamler D, Yadav VN, Dunn P, Al-Holou W, Hollon T, Kim MM, Wahl DR, Camelo-Piragua S, Lieberman AP, Venneti S, McKeever P, Lawrence T, Kurokawa R, Sagher K, Altshuler D, Zhao L, Muraszko K, Castro MG, Lowenstein PR
Lancet Oncol 2023 Sep;24(9):1042-1052. doi: 10.1016/S1470-2045(23)00347-9. PMID: 37657463
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
Epidemiology, molecular classification and WHO grading of ependymoma.
Hübner JM, Kool M, Pfister SM, Pajtler KW
J Neurosurg Sci 2018 Feb;62(1):46-50. Epub 2017 Sep 8 doi: 10.23736/S0390-5616.17.04152-2. PMID: 28895660
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

Combined cytotoxic and immune-stimulatory gene therapy for primary adult high-grade glioma: a phase 1, first-in-human trial.
Umemura Y, Orringer D, Junck L, Varela ML, West MEJ, Faisal SM, Comba A, Heth J, Sagher O, Leung D, Mammoser A, Hervey-Jumper S, Zamler D, Yadav VN, Dunn P, Al-Holou W, Hollon T, Kim MM, Wahl DR, Camelo-Piragua S, Lieberman AP, Venneti S, McKeever P, Lawrence T, Kurokawa R, Sagher K, Altshuler D, Zhao L, Muraszko K, Castro MG, Lowenstein PR
Lancet Oncol 2023 Sep;24(9):1042-1052. doi: 10.1016/S1470-2045(23)00347-9. PMID: 37657463
A Nomogram for Prediction of Patients with Anaplastic Ependymoma in the Brain and Spinal Cord Survival.
Liu H, Sun P
Turk Neurosurg 2023;33(4):626-634. doi: 10.5137/1019-5149.JTN.41103-22.1. PMID: 36622192
Adult Posterior Fossa Anaplastic Ependymoma, Case Series and Literature Review.
Hsu HI, Hsu SS, Chung WY, Yip CM, Liu SH, Liao WC
World Neurosurg 2022 Feb;158:205-209. Epub 2021 Nov 26 doi: 10.1016/j.wneu.2021.11.074. PMID: 34839047
Comparison of Conventional, Diffusion, and Perfusion MRI Between Low-Grade and Anaplastic Extraventricular Ependymoma.
Xing Z, Zhou X, Xiao Z, She D, Wang X, Cao D
AJR Am J Roentgenol 2020 Oct;215(4):978-984. Epub 2020 Aug 18 doi: 10.2214/AJR.20.22764. PMID: 32809860
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

5-ALA fluorescence-guided surgery in pediatric brain tumors-a systematic review.
Schwake M, Schipmann S, Müther M, Köchling M, Brentrup A, Stummer W
Acta Neurochir (Wien) 2019 Jun;161(6):1099-1108. Epub 2019 Apr 13 doi: 10.1007/s00701-019-03898-1. PMID: 30989383

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