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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

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
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

Outcome after treatment of pediatric supratentorial ependymoma: long-term follow-up of a single consecutive institutional series of 26 patients.
Lundar T, Due-Tønnessen BJ, Frič R, Sundseth J, Brandal P, Due-Tønnessen P
Br J Neurosurg 2024 Apr;38(2):514-522. Epub 2021 Jun 7 doi: 10.1080/02688697.2021.1914821. PMID: 34096810
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
Long-term survival following gross total resection of pediatric supratentorial ependymomas without adjuvant therapy.
Tanaka T, Kato N, Hasegawa Y, Nonaka Y, Abe T
Pediatr Neurosurg 2012;48(6):379-84. Epub 2013 Aug 16 doi: 10.1159/000353685. PMID: 23948802

Diagnosis

MRI Phenotype of RELA-fused Pediatric Supratentorial Ependymoma.
Nowak J, Jünger ST, Huflage H, Seidel C, Hohm A, Vandergrift LA, von Hoff K, Rutkowski S, Pietsch T, Warmuth-Metz M
Clin Neuroradiol 2019 Dec;29(4):595-604. Epub 2018 Jul 19 doi: 10.1007/s00062-018-0704-2. PMID: 30027327

Prognosis

Outcome after treatment of pediatric supratentorial ependymoma: long-term follow-up of a single consecutive institutional series of 26 patients.
Lundar T, Due-Tønnessen BJ, Frič R, Sundseth J, Brandal P, Due-Tønnessen P
Br J Neurosurg 2024 Apr;38(2):514-522. Epub 2021 Jun 7 doi: 10.1080/02688697.2021.1914821. PMID: 34096810
MRI Phenotype of RELA-fused Pediatric Supratentorial Ependymoma.
Nowak J, Jünger ST, Huflage H, Seidel C, Hohm A, Vandergrift LA, von Hoff K, Rutkowski S, Pietsch T, Warmuth-Metz M
Clin Neuroradiol 2019 Dec;29(4):595-604. Epub 2018 Jul 19 doi: 10.1007/s00062-018-0704-2. PMID: 30027327
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
Long-term survival following gross total resection of pediatric supratentorial ependymomas without adjuvant therapy.
Tanaka T, Kato N, Hasegawa Y, Nonaka Y, Abe T
Pediatr Neurosurg 2012;48(6):379-84. Epub 2013 Aug 16 doi: 10.1159/000353685. PMID: 23948802

Clinical prediction guides

Outcome after treatment of pediatric supratentorial ependymoma: long-term follow-up of a single consecutive institutional series of 26 patients.
Lundar T, Due-Tønnessen BJ, Frič R, Sundseth J, Brandal P, Due-Tønnessen P
Br J Neurosurg 2024 Apr;38(2):514-522. Epub 2021 Jun 7 doi: 10.1080/02688697.2021.1914821. PMID: 34096810

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