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Cellular ependymoma

MedGen UID:
235137
•Concept ID:
C1384403
•
Neoplastic Process
Synonyms: Cellular Ependymoma; Clear Cell Ependymoma
SNOMED CT: Cellular ependymoma (253067007); Clear cell ependymoma (253068002)
 
Monarch Initiative: MONDO:0003470

Definition

An ependymoma, often supratentorial in location, characterized by the presence of ependymal cells with a perinuclear halo. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCellular 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
Childhood brain tumors: current management, biological insights, and future directions.
Pollack IF, Agnihotri S, Broniscer A
J Neurosurg Pediatr 2019 Mar 1;23(3):261-273. doi: 10.3171/2018.10.PEDS18377. PMID: 30835699Free 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

MRI Characteristics of Spinal Ependymoma in WHO Grade II: A Review of 59 Cases.
Kobayashi K, Ando K, Kato F, Kanemura T, Imagama S, Sato K, Kamiya M, Ito K, Tsushima M, Matsumoto A, Morozumi M, Tanaka S, Machino M, Ishiguro N
Spine (Phila Pa 1976) 2018 May 1;43(9):E525-E530. doi: 10.1097/BRS.0000000000002496. PMID: 29189641
Supratentorial ependymoma in children: to observe or to treat following gross total resection?
Venkatramani R, Dhall G, Patel M, Grimm J, Hawkins C, McComb G, Krieger M, Wong K, O'Neil S, Finlay JL
Pediatr Blood Cancer 2012 Mar;58(3):380-3. Epub 2011 Mar 2 doi: 10.1002/pbc.23086. PMID: 21370439
Histological spectrum of ependymomas and correlation of p53 and Ki-67 expression with ependymoma grade and subtype.
Suri VS, Tatke M, Singh D, Sharma A
Indian J Cancer 2004 Apr-Jun;41(2):66-71. PMID: 15318011

Diagnosis

Fatal holocord recurrence of a pregnancy-related, low-grade spinal ependymoma: case report and review of an unusual clinical phenomenon.
Thakar S, Sivaraju L, Ghosal N, Aryan S, Hegde AS
Spinal Cord Ser Cases 2020 Oct 2;6(1):93. doi: 10.1038/s41394-020-00343-9. PMID: 33009374Free PMC Article
MRI Characteristics of Spinal Ependymoma in WHO Grade II: A Review of 59 Cases.
Kobayashi K, Ando K, Kato F, Kanemura T, Imagama S, Sato K, Kamiya M, Ito K, Tsushima M, Matsumoto A, Morozumi M, Tanaka S, Machino M, Ishiguro N
Spine (Phila Pa 1976) 2018 May 1;43(9):E525-E530. doi: 10.1097/BRS.0000000000002496. PMID: 29189641
Supratentorial ependymoma in children: to observe or to treat following gross total resection?
Venkatramani R, Dhall G, Patel M, Grimm J, Hawkins C, McComb G, Krieger M, Wong K, O'Neil S, Finlay JL
Pediatr Blood Cancer 2012 Mar;58(3):380-3. Epub 2011 Mar 2 doi: 10.1002/pbc.23086. PMID: 21370439
Histological spectrum of ependymomas and correlation of p53 and Ki-67 expression with ependymoma grade and subtype.
Suri VS, Tatke M, Singh D, Sharma A
Indian J Cancer 2004 Apr-Jun;41(2):66-71. PMID: 15318011
Mucin-secreting cellular ependymoma: a light and electron microscopy study.
Cenacchi G, Morra I, Forni M, Giangaspero F
Ultrastruct Pathol 1999 Sep-Oct;23(5):319-23. doi: 10.1080/019131299281464. PMID: 10582269

Therapy

Infantile holocord cellular ependymoma with communicating hydrocephalus: unusual presentation of a rare case.
Aryan S, Ghosal N, Aziz ZA, Hegde AS, Dadlani R
Brain Tumor Pathol 2014 Jan;31(1):47-50. Epub 2013 Mar 20 doi: 10.1007/s10014-013-0145-1. PMID: 23512254
PET findings of intramedullary tumors of the spinal cord using [18F] FDG and [11C] methionine.
Tomura N, Ito Y, Matsuoka H, Saginoya T, Numazawa SI, Mizuno Y, Watanabe K
AJNR Am J Neuroradiol 2013 Jun-Jul;34(6):1278-83. Epub 2012 Dec 28 doi: 10.3174/ajnr.A3374. PMID: 23275592Free PMC Article
Supratentorial ependymoma in children: to observe or to treat following gross total resection?
Venkatramani R, Dhall G, Patel M, Grimm J, Hawkins C, McComb G, Krieger M, Wong K, O'Neil S, Finlay JL
Pediatr Blood Cancer 2012 Mar;58(3):380-3. Epub 2011 Mar 2 doi: 10.1002/pbc.23086. PMID: 21370439

Prognosis

Fatal holocord recurrence of a pregnancy-related, low-grade spinal ependymoma: case report and review of an unusual clinical phenomenon.
Thakar S, Sivaraju L, Ghosal N, Aryan S, Hegde AS
Spinal Cord Ser Cases 2020 Oct 2;6(1):93. doi: 10.1038/s41394-020-00343-9. PMID: 33009374Free PMC Article
MRI Characteristics of Spinal Ependymoma in WHO Grade II: A Review of 59 Cases.
Kobayashi K, Ando K, Kato F, Kanemura T, Imagama S, Sato K, Kamiya M, Ito K, Tsushima M, Matsumoto A, Morozumi M, Tanaka S, Machino M, Ishiguro N
Spine (Phila Pa 1976) 2018 May 1;43(9):E525-E530. doi: 10.1097/BRS.0000000000002496. PMID: 29189641
Infantile holocord cellular ependymoma with communicating hydrocephalus: unusual presentation of a rare case.
Aryan S, Ghosal N, Aziz ZA, Hegde AS, Dadlani R
Brain Tumor Pathol 2014 Jan;31(1):47-50. Epub 2013 Mar 20 doi: 10.1007/s10014-013-0145-1. PMID: 23512254
Histological spectrum of ependymomas and correlation of p53 and Ki-67 expression with ependymoma grade and subtype.
Suri VS, Tatke M, Singh D, Sharma A
Indian J Cancer 2004 Apr-Jun;41(2):66-71. PMID: 15318011
Symptomatic subependymoma. Report of 21 cases with review of the literature.
Scheithauer BW
J Neurosurg 1978 Nov;49(5):689-96. doi: 10.3171/jns.1978.49.5.0689. PMID: 712391

Clinical prediction guides

MRI Characteristics of Spinal Ependymoma in WHO Grade II: A Review of 59 Cases.
Kobayashi K, Ando K, Kato F, Kanemura T, Imagama S, Sato K, Kamiya M, Ito K, Tsushima M, Matsumoto A, Morozumi M, Tanaka S, Machino M, Ishiguro N
Spine (Phila Pa 1976) 2018 May 1;43(9):E525-E530. doi: 10.1097/BRS.0000000000002496. PMID: 29189641
Supratentorial ependymoma in children: to observe or to treat following gross total resection?
Venkatramani R, Dhall G, Patel M, Grimm J, Hawkins C, McComb G, Krieger M, Wong K, O'Neil S, Finlay JL
Pediatr Blood Cancer 2012 Mar;58(3):380-3. Epub 2011 Mar 2 doi: 10.1002/pbc.23086. PMID: 21370439
An unusual variant of ependymoma with extensive tumor cell vacuolization.
Hirato J, Nakazato Y, Iijima M, Yokoo H, Sasaki A, Yokota M, Ono N, Hirato M, Inoue H
Acta Neuropathol 1997 Mar;93(3):310-6. doi: 10.1007/s004010050620. PMID: 9083565

Recent systematic reviews

Factors associated with postoperative outcomes in patients with intramedullary Grade II ependymomas: A Systematic review and meta-analysis.
Sun XY, Wang W, Zhang TT, Kong C, Sun SY, Guo MC, Ding JZ, Lu SB
Medicine (Baltimore) 2019 Jun;98(25):e16185. doi: 10.1097/MD.0000000000016185. PMID: 31232977Free PMC Article

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