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Pediatric supratentorial ependymoma

MedGen UID:
78854
•Concept ID:
C0278650
•
Neoplastic Process
Synonyms: childhood supratentorial ependymoma; Childhood Supratentorial Ependymoma, NOS; Childhood Supratentorial Ependymoma, Not Otherwise Specified; paediatric cerebral ependymoma; paediatric supratentorial ependymoblastoma; pediatric cerebral ependymoma; pediatric supratentorial ependymoblastoma; pediatric supratentorial ependymoma
 
Monarch Initiative: MONDO:0004249

Definition

An ependymoma, not otherwise specified that arises from the supratentorial region of the brain and occurs during childhood. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPediatric supratentorial ependymoma

Professional guidelines

PubMed

Advances in the classification and treatment of pediatric brain tumors.
Fangusaro J, Bandopadhayay P
Curr Opin Pediatr 2021 Feb 1;33(1):26-32. doi: 10.1097/MOP.0000000000000975. PMID: 33278109
Pediatric ependymoma: current treatment and newer therapeutic insights.
Khatua S, Mangum R, Bertrand KC, Zaky W, McCall D, Mack SC
Future Oncol 2018 Dec;14(30):3175-3186. Epub 2018 Nov 12 doi: 10.2217/fon-2018-0502. PMID: 30418040
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

Updates in the classification of ependymal neoplasms: The 2021 WHO Classification and beyond.
Kresbach C, Neyazi S, Schüller U
Brain Pathol 2022 Jul;32(4):e13068. Epub 2022 Mar 21 doi: 10.1111/bpa.13068. PMID: 35307892Free PMC Article
Ependymal Tumors.
Santi M, Viaene AN, Hawkins C
Pediatr Dev Pathol 2022 Jan-Feb;25(1):59-67. doi: 10.1177/10935266211018928. PMID: 35168420
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
Pediatric ependymoma: current treatment and newer therapeutic insights.
Khatua S, Mangum R, Bertrand KC, Zaky W, McCall D, Mack SC
Future Oncol 2018 Dec;14(30):3175-3186. Epub 2018 Nov 12 doi: 10.2217/fon-2018-0502. PMID: 30418040
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

Ependymal Tumors.
Santi M, Viaene AN, Hawkins C
Pediatr Dev Pathol 2022 Jan-Feb;25(1):59-67. doi: 10.1177/10935266211018928. PMID: 35168420
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
Pediatric ependymoma: current treatment and newer therapeutic insights.
Khatua S, Mangum R, Bertrand KC, Zaky W, McCall D, Mack SC
Future Oncol 2018 Dec;14(30):3175-3186. Epub 2018 Nov 12 doi: 10.2217/fon-2018-0502. PMID: 30418040
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
Supratentorial Tumors in Pediatric Patients.
Zamora C, Huisman TA, Izbudak I
Neuroimaging Clin N Am 2017 Feb;27(1):39-67. doi: 10.1016/j.nic.2016.08.003. PMID: 27889023

Therapy

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
Pediatric ependymoma: current treatment and newer therapeutic insights.
Khatua S, Mangum R, Bertrand KC, Zaky W, McCall D, Mack SC
Future Oncol 2018 Dec;14(30):3175-3186. Epub 2018 Nov 12 doi: 10.2217/fon-2018-0502. PMID: 30418040
Current therapy and the evolving molecular landscape of paediatric ependymoma.
Khatua S, Ramaswamy V, Bouffet E
Eur J Cancer 2017 Jan;70:34-41. Epub 2016 Nov 18 doi: 10.1016/j.ejca.2016.10.013. PMID: 27866097
High-grade gliomas in children.
Cage TA, Mueller S, Haas-Kogan D, Gupta N
Neurosurg Clin N Am 2012 Jul;23(3):515-23. Epub 2012 Jun 5 doi: 10.1016/j.nec.2012.04.007. PMID: 22748663
Pediatric glial tumors.
Cohen KJ, Broniscer A, Glod J
Curr Treat Options Oncol 2001 Dec;2(6):529-36. doi: 10.1007/s11864-001-0074-9. PMID: 12057098

Prognosis

Updates in the classification of ependymal neoplasms: The 2021 WHO Classification and beyond.
Kresbach C, Neyazi S, Schüller U
Brain Pathol 2022 Jul;32(4):e13068. Epub 2022 Mar 21 doi: 10.1111/bpa.13068. PMID: 35307892Free PMC Article
Ependymal Tumors.
Santi M, Viaene AN, Hawkins C
Pediatr Dev Pathol 2022 Jan-Feb;25(1):59-67. doi: 10.1177/10935266211018928. PMID: 35168420
Pediatric Supratentorial Ependymoma: Surgical, Clinical, and Molecular Analysis.
Lillard JC, Venable GT, Khan NR, Tatevossian RG, Dalton J, Vaughn BN, Klimo P
Neurosurgery 2019 Jul 1;85(1):41-49. doi: 10.1093/neuros/nyy239. PMID: 29917116
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

Updates in the classification of ependymal neoplasms: The 2021 WHO Classification and beyond.
Kresbach C, Neyazi S, Schüller U
Brain Pathol 2022 Jul;32(4):e13068. Epub 2022 Mar 21 doi: 10.1111/bpa.13068. PMID: 35307892Free PMC Article
Supratentorial ependymoma in childhood: more than just RELA or YAP.
Zschernack V, Jünger ST, Mynarek M, Rutkowski S, Garre ML, Ebinger M, Neu M, Faber J, Erdlenbruch B, Claviez A, Bielack S, Brozou T, Frühwald MC, Dörner E, Dreschmann V, Stock A, Solymosi L, Hench J, Frank S, Vokuhl C, Waha A, Andreiuolo F, Pietsch T
Acta Neuropathol 2021 Mar;141(3):455-466. Epub 2021 Jan 22 doi: 10.1007/s00401-020-02260-5. PMID: 33481105Free 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
Current therapy and the evolving molecular landscape of paediatric ependymoma.
Khatua S, Ramaswamy V, Bouffet E
Eur J Cancer 2017 Jan;70:34-41. Epub 2016 Nov 18 doi: 10.1016/j.ejca.2016.10.013. PMID: 27866097
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

Recent systematic reviews

A systematic review and meta-analysis of outcomes in pediatric, recurrent ependymoma.
Byer L, Kline CN, Coleman C, Allen IE, Whitaker E, Mueller S
J Neurooncol 2019 Sep;144(3):445-452. Epub 2019 Sep 9 doi: 10.1007/s11060-019-03255-3. PMID: 31502040
A systematic review of treatment outcomes in pediatric patients with intracranial ependymomas.
Cage TA, Clark AJ, Aranda D, Gupta N, Sun PP, Parsa AT, Auguste KI
J Neurosurg Pediatr 2013 Jun;11(6):673-81. Epub 2013 Mar 29 doi: 10.3171/2013.2.PEDS12345. PMID: 23540528
How specific is the MRI appearance of supratentorial atypical teratoid rhabdoid tumors?
Au Yong KJ, Jaremko JL, Jans L, Bhargava R, Coleman LT, Mehta V, Ditchfield MR
Pediatr Radiol 2013 Mar;43(3):347-54. Epub 2012 Nov 10 doi: 10.1007/s00247-012-2530-z. PMID: 23143401

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