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Epithelioid Glioblastoma

MedGen UID:
927112
•Concept ID:
C4289580
•
Neoplastic Process
Synonym: Epithelioid glioblastoma
SNOMED CT: Epithelioid glioblastoma (733837004)

Definition

An IDH-wildtype glioblastoma characterized by the presence of large epithelioid cells with abundant eosinophilic cytoplasm, vesicular chromatin, and prominent nucleoli. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVEpithelioid Glioblastoma

Professional guidelines

PubMed

Clinicopathological characteristics and treatment outcomes of epithelioid glioblastoma.
Sun K, Zhou X, Li T, Zuo M, Li J, Liu Y
Neurosurg Rev 2021 Dec;44(6):3335-3348. Epub 2021 Feb 17 doi: 10.1007/s10143-021-01492-7. PMID: 33598819
Confirming Diagnosis and Effective Treatment for Rare Epithelioid Glioblastoma Variant: An Integrated Survival Analysis of the Literature.
Lu VM, George ND, Brown DA, Akinduro OO, Raghunathan A, Jentoft M, Quinones-Hinojosa A, Chaichana KL
World Neurosurg 2019 Nov;131:243-251.e2. Epub 2019 Aug 9 doi: 10.1016/j.wneu.2019.08.007. PMID: 31404694
If it is Not a Glioblastoma, Then What is it? A Differential Diagnostic Review.
Gokden M
Adv Anat Pathol 2017 Nov;24(6):379-391. doi: 10.1097/PAP.0000000000000170. PMID: 28885262

Recent clinical studies

Etiology

Systematic Review and Individual Patient Data Analysis of Uncommon Variants of Glioblastoma: An Analysis of 196 Cases.
Mallick S, Benson R, Venkatesulu B, Melgandi W, Rath GK
Neurol India 2022 Sep-Oct;70(5):2086-2092. doi: 10.4103/0028-3886.359222. PMID: 36352613
Glioblastoma: Changing concepts in the WHO CNS5 classification.
Chen J, Han P, Dahiya S
Indian J Pathol Microbiol 2022 May;65(Supplement):S24-S32. doi: 10.4103/ijpm.ijpm_1109_21. PMID: 35562131
Analysis of 24 cases of epithelioid glioblastoma: Experience from a tertiary centre of North India.
Chatterjee D, Radotra BD, Aggarwal D, Madan R, Gupta SK
Ann Diagn Pathol 2021 Feb;50:151679. Epub 2020 Dec 13 doi: 10.1016/j.anndiagpath.2020.151679. PMID: 33341703
Evaluation of EZH2 expression, BRAF V600E mutation, and CDKN2A/B deletions in epithelioid glioblastoma and anaplastic pleomorphic xanthoastrocytoma.
Wang J, Liu Z, Cui Y, Liu Y, Fang J, Xu L, He Y, Du J, Su Y, Zou W, Xu Z, Li G
J Neurooncol 2019 Aug;144(1):137-146. Epub 2019 Jun 18 doi: 10.1007/s11060-019-03212-0. PMID: 31214915
If it is Not a Glioblastoma, Then What is it? A Differential Diagnostic Review.
Gokden M
Adv Anat Pathol 2017 Nov;24(6):379-391. doi: 10.1097/PAP.0000000000000170. PMID: 28885262

Diagnosis

Glioblastoma: Changing concepts in the WHO CNS5 classification.
Chen J, Han P, Dahiya S
Indian J Pathol Microbiol 2022 May;65(Supplement):S24-S32. doi: 10.4103/ijpm.ijpm_1109_21. PMID: 35562131
Clinicopathological characteristics and treatment outcomes of epithelioid glioblastoma.
Sun K, Zhou X, Li T, Zuo M, Li J, Liu Y
Neurosurg Rev 2021 Dec;44(6):3335-3348. Epub 2021 Feb 17 doi: 10.1007/s10143-021-01492-7. PMID: 33598819
BRAF alterations in primary brain tumors.
Maraka S, Janku F
Discov Med 2018 Aug;26(141):51-60. PMID: 30265855
Clinicopathological and genetic association between epithelioid glioblastoma and pleomorphic xanthoastrocytoma.
Furuta T, Miyoshi H, Komaki S, Arakawa F, Morioka M, Ohshima K, Nakada M, Sugita Y
Neuropathology 2018 Jun;38(3):218-227. Epub 2018 Mar 13 doi: 10.1111/neup.12459. PMID: 29532523
If it is Not a Glioblastoma, Then What is it? A Differential Diagnostic Review.
Gokden M
Adv Anat Pathol 2017 Nov;24(6):379-391. doi: 10.1097/PAP.0000000000000170. PMID: 28885262

Therapy

Systematic Review and Individual Patient Data Analysis of Uncommon Variants of Glioblastoma: An Analysis of 196 Cases.
Mallick S, Benson R, Venkatesulu B, Melgandi W, Rath GK
Neurol India 2022 Sep-Oct;70(5):2086-2092. doi: 10.4103/0028-3886.359222. PMID: 36352613
Response to BRAF and MEK1/2 inhibition in a young adult with BRAF V600E mutant epithelioid glioblastoma multiforme: A Case Report and Literature Review.
Venkatesh A, Joshi A, Allinson K, Das T, Santarius T, Jefferies SJ, Harris FP, Jena R, Doherty GJ
Curr Probl Cancer 2021 Oct;45(5):100701. Epub 2021 Jan 12 doi: 10.1016/j.currproblcancer.2020.100701. PMID: 33461766
Epithelioid glioblastoma with microglia features: potential for novel therapy.
Nakagomi N, Sakamoto D, Hirose T, Takagi T, Murase M, Nakagomi T, Yoshimura S, Hirota S
Brain Pathol 2020 Nov;30(6):1119-1133. Epub 2020 Aug 6 doi: 10.1111/bpa.12887. PMID: 32687679Free PMC Article
Dramatic response of BRAF V600E-mutant epithelioid glioblastoma to combination therapy with BRAF and MEK inhibitor: establishment and xenograft of a cell line to predict clinical efficacy.
Kanemaru Y, Natsumeda M, Okada M, Saito R, Kobayashi D, Eda T, Watanabe J, Saito S, Tsukamoto Y, Oishi M, Saito H, Nagahashi M, Sasaki T, Hashizume R, Aoyama H, Wakai T, Kakita A, Fujii Y
Acta Neuropathol Commun 2019 Jul 25;7(1):119. doi: 10.1186/s40478-019-0774-7. PMID: 31345255Free PMC Article
Concurrent TERT promoter and BRAF V600E mutation in epithelioid glioblastoma and concomitant low-grade astrocytoma.
Matsumura N, Nakajima N, Yamazaki T, Nagano T, Kagoshima K, Nobusawa S, Ikota H, Yokoo H
Neuropathology 2017 Feb;37(1):58-63. Epub 2016 Jun 15 doi: 10.1111/neup.12318. PMID: 27302309

Prognosis

Adult epithelioid glioblastoma exhibits an extremely poor prognosis and high frequency of SWI/SNF complex mutation: Insights from a retrospective study.
Xi S, Jiang S, Li H, Huang Q, Lu J, Zhang X, Li Z, Zeng J
Int J Cancer 2024 Jul 1;155(1):172-183. Epub 2024 Feb 27 doi: 10.1002/ijc.34854. PMID: 38411299
Clinicopathological characteristics and treatment outcomes of epithelioid glioblastoma.
Sun K, Zhou X, Li T, Zuo M, Li J, Liu Y
Neurosurg Rev 2021 Dec;44(6):3335-3348. Epub 2021 Feb 17 doi: 10.1007/s10143-021-01492-7. PMID: 33598819
Immunohistochemical and molecular genetic study on epithelioid glioblastoma: Series of seven cases with review of literature.
Khanna G, Pathak P, Suri V, Sharma MC, Chaturvedi S, Ahuja A, Bhardwaj M, Garg A, Sarkar C, Sharma R
Pathol Res Pract 2018 May;214(5):679-685. Epub 2018 Mar 22 doi: 10.1016/j.prp.2018.03.019. PMID: 29615337
If it is Not a Glioblastoma, Then What is it? A Differential Diagnostic Review.
Gokden M
Adv Anat Pathol 2017 Nov;24(6):379-391. doi: 10.1097/PAP.0000000000000170. PMID: 28885262
Epithelioid/rhabdoid glioblastoma: a highly aggressive subtype of glioblastoma.
Sugimoto K, Ideguchi M, Kimura T, Kajiwara K, Imoto H, Sadahiro H, Ishii A, Kawano H, Ikeda E, Suzuki M
Brain Tumor Pathol 2016 Apr;33(2):137-46. Epub 2015 Dec 14 doi: 10.1007/s10014-015-0243-3. PMID: 26667174

Clinical prediction guides

Analysis of 24 cases of epithelioid glioblastoma: Experience from a tertiary centre of North India.
Chatterjee D, Radotra BD, Aggarwal D, Madan R, Gupta SK
Ann Diagn Pathol 2021 Feb;50:151679. Epub 2020 Dec 13 doi: 10.1016/j.anndiagpath.2020.151679. PMID: 33341703
Confirming Diagnosis and Effective Treatment for Rare Epithelioid Glioblastoma Variant: An Integrated Survival Analysis of the Literature.
Lu VM, George ND, Brown DA, Akinduro OO, Raghunathan A, Jentoft M, Quinones-Hinojosa A, Chaichana KL
World Neurosurg 2019 Nov;131:243-251.e2. Epub 2019 Aug 9 doi: 10.1016/j.wneu.2019.08.007. PMID: 31404694
Anaplastic ganglioglioma with epithelioid cell components.
Murakami C, Yamazaki T, Shintoku R, Aihara M, Yoshimoto Y, Matsumura N, Nobusawa S, Ikota H, Hirato J, Yokoo H
Neuropathology 2018 Oct;38(5):498-502. Epub 2018 Jun 4 doi: 10.1111/neup.12479. PMID: 29869356
Clinicopathological and genetic association between epithelioid glioblastoma and pleomorphic xanthoastrocytoma.
Furuta T, Miyoshi H, Komaki S, Arakawa F, Morioka M, Ohshima K, Nakada M, Sugita Y
Neuropathology 2018 Jun;38(3):218-227. Epub 2018 Mar 13 doi: 10.1111/neup.12459. PMID: 29532523
If it is Not a Glioblastoma, Then What is it? A Differential Diagnostic Review.
Gokden M
Adv Anat Pathol 2017 Nov;24(6):379-391. doi: 10.1097/PAP.0000000000000170. PMID: 28885262

Recent systematic reviews

The Significance of BRAF Mutation in the Epithelioid Glioblastoma Subtype: A Systematic Literature Review and a Case Report with a Unique Intraventricular Topography.
Prieto R, Barrios L, Ebrat-Mancilla E, Martín P, Tejerina E
Int J Surg Pathol 2024 Jun;32(4):649-666. Epub 2023 Sep 24 doi: 10.1177/10668969231195026. PMID: 37743598
Systematic Review and Individual Patient Data Analysis of Uncommon Variants of Glioblastoma: An Analysis of 196 Cases.
Mallick S, Benson R, Venkatesulu B, Melgandi W, Rath GK
Neurol India 2022 Sep-Oct;70(5):2086-2092. doi: 10.4103/0028-3886.359222. PMID: 36352613
Prevalence of BRAFV600 in glioma and use of BRAF Inhibitors in patients with BRAFV600 mutation-positive glioma: systematic review.
Andrews LJ, Thornton ZA, Saincher SS, Yao IY, Dawson S, McGuinness LA, Jones HE, Jefferies S, Short SC, Cheng HY, McAleenan A, Higgins JPT, Kurian KM
Neuro Oncol 2022 Apr 1;24(4):528-540. doi: 10.1093/neuonc/noab247. PMID: 34718782Free PMC Article
Confirming Diagnosis and Effective Treatment for Rare Epithelioid Glioblastoma Variant: An Integrated Survival Analysis of the Literature.
Lu VM, George ND, Brown DA, Akinduro OO, Raghunathan A, Jentoft M, Quinones-Hinojosa A, Chaichana KL
World Neurosurg 2019 Nov;131:243-251.e2. Epub 2019 Aug 9 doi: 10.1016/j.wneu.2019.08.007. PMID: 31404694
Clinical relevance of BRAF status in glial and glioneuronal tumors: A systematic review.
Sugiura Y, Nagaishi M
J Clin Neurosci 2019 Aug;66:196-201. Epub 2019 May 27 doi: 10.1016/j.jocn.2019.05.014. PMID: 31147232

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