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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

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
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
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

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
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
Ovarian ependymoma.
Erdoğan G, Ozel E, Peştereli HE, Salar Z, Tirak B, Karaveli S
APMIS 2005 Apr;113(4):301-3. doi: 10.1111/j.1600-0463.2005.apm_10.x. PMID: 15865613

Therapy

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
Bicentric Treatment Outcomes After Proton Therapy for Nonmyxopapillary High-Grade Spinal Cord Ependymoma in Children.
Indelicato DJ, Ioakeim-Ioannidou M, Grippin AJ, Bradley JA, Mailhot Vega RB, Viviers E, Tarbell NJ, Yock TI, MacDonald SM
Int J Radiat Oncol Biol Phys 2022 Feb 1;112(2):335-341. Epub 2021 Sep 29 doi: 10.1016/j.ijrobp.2021.09.030. PMID: 34597719
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
Intracavitary chemotherapy (Gliadel) and oral low-dose etoposide for recurrent anaplastic ependymoma.
Sardi I, Sanzo M, Giordano F, Sandri A, Mussa F, Donati PA, Genitori L
Oncol Rep 2008 May;19(5):1219-23. PMID: 18425379

Prognosis

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
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
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

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
Ovarian ependymoma.
Erdoğan G, Ozel E, Peştereli HE, Salar Z, Tirak B, Karaveli S
APMIS 2005 Apr;113(4):301-3. doi: 10.1111/j.1600-0463.2005.apm_10.x. PMID: 15865613

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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