Confirming Diagnosis and Effective Treatment for Rare Epithelioid Glioblastoma Variant: An Integrated Survival Analysis of the Literature

Victor M. Lu, Naveen D. George, Desmond A. Brown, Oluwaseun O. Akinduro, Aditya Raghunathan, Mark Jentoft, Alfredo Quinones-Hinojosa, Kaisorn L. Chaichana

Research output: Contribution to journalReview article

Abstract

Background: Epithelioid glioblastoma (eGBM) is a very rare histologic variant of glioblastoma that has not been studied in isolation and, therefore, its optimal management has been largely assumed, but not confirmed. The aim of this study was to analyze all reported cases describing the presentation and clinical features to better understand the clinical significance of this histologic diagnosis. Methods: A comprehensive literature search was conducted from 2005 to April 2019 identifying cases of eGBM that satisfied selection criteria for analysis. Survival was investigated using Kaplan-Meier estimations, and then univariate and multivariate logistic regression analyses for primary end point overall survival (OS) and second end point progression-free survival (PFS). Results: A total cohort of 59 eGBM cases from 28 articles were included for final analysis. Median age of patients at diagnosis was 30 years, with 29 (46%) female patients. When reported, 100% (37/37) cases were IDH1-wild-type and 63% (19/30) were positive for the BRAF V600E mutation by immunohistochemistry. Median OS and PFS were estimated to be 11.0 months (95% confidence interval, 6.5–13.0) and 7.0 months (95% confidence interval, 3.0–10.0), respectively. Surgical extent of resection, radiation therapy, and chemotherapy all predicted superior OS and PFS on multivariate analysis (P < 0.05). No biomarkers prognosticated survival. Conclusions: These findings indicate that the histologic diagnosis of eGBM does not deviate from the clinical course of the broader glioblastoma diagnosis, despite being a unique histologic identity. These results argue against the temptation to deviate from the traditional management paradigm of surgery, radiation, and chemotherapy for glioblastoma based on this histology alone.

Original languageEnglish (US)
JournalWorld neurosurgery
DOIs
StateAccepted/In press - Jan 1 2019

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Glioblastoma
Survival Analysis
Survival
Disease-Free Survival
Therapeutics
Confidence Intervals
Drug Therapy
Patient Selection
Histology
Radiotherapy
Multivariate Analysis
Biomarkers
Logistic Models
Immunohistochemistry
Regression Analysis
Radiation
Mutation

Keywords

  • BRAF
  • Epithelioid
  • GBM
  • Glioblastoma
  • INI-1
  • Rhabdoid

ASJC Scopus subject areas

  • Surgery
  • Clinical Neurology

Cite this

Confirming Diagnosis and Effective Treatment for Rare Epithelioid Glioblastoma Variant : An Integrated Survival Analysis of the Literature. / Lu, Victor M.; George, Naveen D.; Brown, Desmond A.; Akinduro, Oluwaseun O.; Raghunathan, Aditya; Jentoft, Mark; Quinones-Hinojosa, Alfredo; Chaichana, Kaisorn L.

In: World neurosurgery, 01.01.2019.

Research output: Contribution to journalReview article

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title = "Confirming Diagnosis and Effective Treatment for Rare Epithelioid Glioblastoma Variant: An Integrated Survival Analysis of the Literature",
abstract = "Background: Epithelioid glioblastoma (eGBM) is a very rare histologic variant of glioblastoma that has not been studied in isolation and, therefore, its optimal management has been largely assumed, but not confirmed. The aim of this study was to analyze all reported cases describing the presentation and clinical features to better understand the clinical significance of this histologic diagnosis. Methods: A comprehensive literature search was conducted from 2005 to April 2019 identifying cases of eGBM that satisfied selection criteria for analysis. Survival was investigated using Kaplan-Meier estimations, and then univariate and multivariate logistic regression analyses for primary end point overall survival (OS) and second end point progression-free survival (PFS). Results: A total cohort of 59 eGBM cases from 28 articles were included for final analysis. Median age of patients at diagnosis was 30 years, with 29 (46{\%}) female patients. When reported, 100{\%} (37/37) cases were IDH1-wild-type and 63{\%} (19/30) were positive for the BRAF V600E mutation by immunohistochemistry. Median OS and PFS were estimated to be 11.0 months (95{\%} confidence interval, 6.5–13.0) and 7.0 months (95{\%} confidence interval, 3.0–10.0), respectively. Surgical extent of resection, radiation therapy, and chemotherapy all predicted superior OS and PFS on multivariate analysis (P < 0.05). No biomarkers prognosticated survival. Conclusions: These findings indicate that the histologic diagnosis of eGBM does not deviate from the clinical course of the broader glioblastoma diagnosis, despite being a unique histologic identity. These results argue against the temptation to deviate from the traditional management paradigm of surgery, radiation, and chemotherapy for glioblastoma based on this histology alone.",
keywords = "BRAF, Epithelioid, GBM, Glioblastoma, INI-1, Rhabdoid",
author = "Lu, {Victor M.} and George, {Naveen D.} and Brown, {Desmond A.} and Akinduro, {Oluwaseun O.} and Aditya Raghunathan and Mark Jentoft and Alfredo Quinones-Hinojosa and Chaichana, {Kaisorn L.}",
year = "2019",
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T1 - Confirming Diagnosis and Effective Treatment for Rare Epithelioid Glioblastoma Variant

T2 - An Integrated Survival Analysis of the Literature

AU - Lu, Victor M.

AU - George, Naveen D.

AU - Brown, Desmond A.

AU - Akinduro, Oluwaseun O.

AU - Raghunathan, Aditya

AU - Jentoft, Mark

AU - Quinones-Hinojosa, Alfredo

AU - Chaichana, Kaisorn L.

PY - 2019/1/1

Y1 - 2019/1/1

N2 - Background: Epithelioid glioblastoma (eGBM) is a very rare histologic variant of glioblastoma that has not been studied in isolation and, therefore, its optimal management has been largely assumed, but not confirmed. The aim of this study was to analyze all reported cases describing the presentation and clinical features to better understand the clinical significance of this histologic diagnosis. Methods: A comprehensive literature search was conducted from 2005 to April 2019 identifying cases of eGBM that satisfied selection criteria for analysis. Survival was investigated using Kaplan-Meier estimations, and then univariate and multivariate logistic regression analyses for primary end point overall survival (OS) and second end point progression-free survival (PFS). Results: A total cohort of 59 eGBM cases from 28 articles were included for final analysis. Median age of patients at diagnosis was 30 years, with 29 (46%) female patients. When reported, 100% (37/37) cases were IDH1-wild-type and 63% (19/30) were positive for the BRAF V600E mutation by immunohistochemistry. Median OS and PFS were estimated to be 11.0 months (95% confidence interval, 6.5–13.0) and 7.0 months (95% confidence interval, 3.0–10.0), respectively. Surgical extent of resection, radiation therapy, and chemotherapy all predicted superior OS and PFS on multivariate analysis (P < 0.05). No biomarkers prognosticated survival. Conclusions: These findings indicate that the histologic diagnosis of eGBM does not deviate from the clinical course of the broader glioblastoma diagnosis, despite being a unique histologic identity. These results argue against the temptation to deviate from the traditional management paradigm of surgery, radiation, and chemotherapy for glioblastoma based on this histology alone.

AB - Background: Epithelioid glioblastoma (eGBM) is a very rare histologic variant of glioblastoma that has not been studied in isolation and, therefore, its optimal management has been largely assumed, but not confirmed. The aim of this study was to analyze all reported cases describing the presentation and clinical features to better understand the clinical significance of this histologic diagnosis. Methods: A comprehensive literature search was conducted from 2005 to April 2019 identifying cases of eGBM that satisfied selection criteria for analysis. Survival was investigated using Kaplan-Meier estimations, and then univariate and multivariate logistic regression analyses for primary end point overall survival (OS) and second end point progression-free survival (PFS). Results: A total cohort of 59 eGBM cases from 28 articles were included for final analysis. Median age of patients at diagnosis was 30 years, with 29 (46%) female patients. When reported, 100% (37/37) cases were IDH1-wild-type and 63% (19/30) were positive for the BRAF V600E mutation by immunohistochemistry. Median OS and PFS were estimated to be 11.0 months (95% confidence interval, 6.5–13.0) and 7.0 months (95% confidence interval, 3.0–10.0), respectively. Surgical extent of resection, radiation therapy, and chemotherapy all predicted superior OS and PFS on multivariate analysis (P < 0.05). No biomarkers prognosticated survival. Conclusions: These findings indicate that the histologic diagnosis of eGBM does not deviate from the clinical course of the broader glioblastoma diagnosis, despite being a unique histologic identity. These results argue against the temptation to deviate from the traditional management paradigm of surgery, radiation, and chemotherapy for glioblastoma based on this histology alone.

KW - BRAF

KW - Epithelioid

KW - GBM

KW - Glioblastoma

KW - INI-1

KW - Rhabdoid

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DO - 10.1016/j.wneu.2019.08.007

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