Gliomatosis Cerebri Among Children and Adolescents

An Individual-Patient Data Meta-analysis of 182 Patients

Marios K. Georgakis, Georgios Tsivgoulis, Apostolos Pourtsidis, Eleni Th Petridou

Research output: Contribution to journalArticle

Abstract

Background: Gliomatosis cerebri is a rare but fatal widespread infiltrating central nervous system tumor. We aimed to describe diagnostic and prognostic features of gliomatosis cerebri among children and adolescents. Methods: We conducted a systematic literature review for published case reports and case series on patients with histologically confirmed gliomatosis cerebri and extracted data on an individual patient level for those aged 0-18 years. Multivariable Cox proportional hazard models were fit for overall survival. Results: Following screening of 274 published studies, 182 gliomatosis cerebri patients (63% males) aged 0-18 years with individual-level data available were identified. The most common presenting symptoms were seizures (52%), focal motor deficits (36%), and headache (30%). Imaging showed bilateral hemisphere involvement in 60%, infratentorial infiltration in 39%, and a focal contrast-enhanced mass (type II gliomatosis cerebri) in 27% of cases. Anaplastic astrocytoma was the most common histologic subtype of pediatric gliomatosis cerebri, whereas MGMT promoter methylation, IDH1 mutations, and codeletion of 1p/19q were less common molecular aberrations, as compared to adult gliomatosis cerebri. In the multivariable analyses, age at diagnosis >4 years, extended central nervous system infiltration, coordination abnormalities, and cognitive decline were predictors of worse outcome. Conversely, IDH1 mutations were associated with prolonged overall survival. Chemotherapy and extended surgical resection were associated with improved outcome, whereas radiotherapy was not associated with overall survival and was inferior to chemotherapy alone. Conclusion: Gliomatosis cerebri among children and adolescents presents distinct histopathologic and molecular features compared to adults. However, similar associations of chemotherapy, and, when feasible, extended surgical resection, with favorable outcomes were noted among the 2 age groups.

Original languageEnglish (US)
Pages (from-to)394-401
Number of pages8
JournalJournal of child neurology
Volume34
Issue number7
DOIs
StatePublished - Jun 1 2019

Fingerprint

Neuroepithelial Neoplasms
Meta-Analysis
Drug Therapy
Survival
Central Nervous System Neoplasms
Mutation
Astrocytoma
Proportional Hazards Models
Methylation
Headache
Seizures
Radiotherapy
Central Nervous System
Age Groups
Pediatrics

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology

Cite this

Gliomatosis Cerebri Among Children and Adolescents : An Individual-Patient Data Meta-analysis of 182 Patients. / Georgakis, Marios K.; Tsivgoulis, Georgios; Pourtsidis, Apostolos; Petridou, Eleni Th.

In: Journal of child neurology, Vol. 34, No. 7, 01.06.2019, p. 394-401.

Research output: Contribution to journalArticle

Georgakis, Marios K. ; Tsivgoulis, Georgios ; Pourtsidis, Apostolos ; Petridou, Eleni Th. / Gliomatosis Cerebri Among Children and Adolescents : An Individual-Patient Data Meta-analysis of 182 Patients. In: Journal of child neurology. 2019 ; Vol. 34, No. 7. pp. 394-401.
@article{5b0cb36de8bc4304bcfd3528154cf375,
title = "Gliomatosis Cerebri Among Children and Adolescents: An Individual-Patient Data Meta-analysis of 182 Patients",
abstract = "Background: Gliomatosis cerebri is a rare but fatal widespread infiltrating central nervous system tumor. We aimed to describe diagnostic and prognostic features of gliomatosis cerebri among children and adolescents. Methods: We conducted a systematic literature review for published case reports and case series on patients with histologically confirmed gliomatosis cerebri and extracted data on an individual patient level for those aged 0-18 years. Multivariable Cox proportional hazard models were fit for overall survival. Results: Following screening of 274 published studies, 182 gliomatosis cerebri patients (63{\%} males) aged 0-18 years with individual-level data available were identified. The most common presenting symptoms were seizures (52{\%}), focal motor deficits (36{\%}), and headache (30{\%}). Imaging showed bilateral hemisphere involvement in 60{\%}, infratentorial infiltration in 39{\%}, and a focal contrast-enhanced mass (type II gliomatosis cerebri) in 27{\%} of cases. Anaplastic astrocytoma was the most common histologic subtype of pediatric gliomatosis cerebri, whereas MGMT promoter methylation, IDH1 mutations, and codeletion of 1p/19q were less common molecular aberrations, as compared to adult gliomatosis cerebri. In the multivariable analyses, age at diagnosis >4 years, extended central nervous system infiltration, coordination abnormalities, and cognitive decline were predictors of worse outcome. Conversely, IDH1 mutations were associated with prolonged overall survival. Chemotherapy and extended surgical resection were associated with improved outcome, whereas radiotherapy was not associated with overall survival and was inferior to chemotherapy alone. Conclusion: Gliomatosis cerebri among children and adolescents presents distinct histopathologic and molecular features compared to adults. However, similar associations of chemotherapy, and, when feasible, extended surgical resection, with favorable outcomes were noted among the 2 age groups.",
author = "Georgakis, {Marios K.} and Georgios Tsivgoulis and Apostolos Pourtsidis and Petridou, {Eleni Th}",
year = "2019",
month = "6",
day = "1",
doi = "10.1177/0883073819836551",
language = "English (US)",
volume = "34",
pages = "394--401",
journal = "Journal of Child Neurology",
issn = "0883-0738",
publisher = "SAGE Publications Inc.",
number = "7",

}

TY - JOUR

T1 - Gliomatosis Cerebri Among Children and Adolescents

T2 - An Individual-Patient Data Meta-analysis of 182 Patients

AU - Georgakis, Marios K.

AU - Tsivgoulis, Georgios

AU - Pourtsidis, Apostolos

AU - Petridou, Eleni Th

PY - 2019/6/1

Y1 - 2019/6/1

N2 - Background: Gliomatosis cerebri is a rare but fatal widespread infiltrating central nervous system tumor. We aimed to describe diagnostic and prognostic features of gliomatosis cerebri among children and adolescents. Methods: We conducted a systematic literature review for published case reports and case series on patients with histologically confirmed gliomatosis cerebri and extracted data on an individual patient level for those aged 0-18 years. Multivariable Cox proportional hazard models were fit for overall survival. Results: Following screening of 274 published studies, 182 gliomatosis cerebri patients (63% males) aged 0-18 years with individual-level data available were identified. The most common presenting symptoms were seizures (52%), focal motor deficits (36%), and headache (30%). Imaging showed bilateral hemisphere involvement in 60%, infratentorial infiltration in 39%, and a focal contrast-enhanced mass (type II gliomatosis cerebri) in 27% of cases. Anaplastic astrocytoma was the most common histologic subtype of pediatric gliomatosis cerebri, whereas MGMT promoter methylation, IDH1 mutations, and codeletion of 1p/19q were less common molecular aberrations, as compared to adult gliomatosis cerebri. In the multivariable analyses, age at diagnosis >4 years, extended central nervous system infiltration, coordination abnormalities, and cognitive decline were predictors of worse outcome. Conversely, IDH1 mutations were associated with prolonged overall survival. Chemotherapy and extended surgical resection were associated with improved outcome, whereas radiotherapy was not associated with overall survival and was inferior to chemotherapy alone. Conclusion: Gliomatosis cerebri among children and adolescents presents distinct histopathologic and molecular features compared to adults. However, similar associations of chemotherapy, and, when feasible, extended surgical resection, with favorable outcomes were noted among the 2 age groups.

AB - Background: Gliomatosis cerebri is a rare but fatal widespread infiltrating central nervous system tumor. We aimed to describe diagnostic and prognostic features of gliomatosis cerebri among children and adolescents. Methods: We conducted a systematic literature review for published case reports and case series on patients with histologically confirmed gliomatosis cerebri and extracted data on an individual patient level for those aged 0-18 years. Multivariable Cox proportional hazard models were fit for overall survival. Results: Following screening of 274 published studies, 182 gliomatosis cerebri patients (63% males) aged 0-18 years with individual-level data available were identified. The most common presenting symptoms were seizures (52%), focal motor deficits (36%), and headache (30%). Imaging showed bilateral hemisphere involvement in 60%, infratentorial infiltration in 39%, and a focal contrast-enhanced mass (type II gliomatosis cerebri) in 27% of cases. Anaplastic astrocytoma was the most common histologic subtype of pediatric gliomatosis cerebri, whereas MGMT promoter methylation, IDH1 mutations, and codeletion of 1p/19q were less common molecular aberrations, as compared to adult gliomatosis cerebri. In the multivariable analyses, age at diagnosis >4 years, extended central nervous system infiltration, coordination abnormalities, and cognitive decline were predictors of worse outcome. Conversely, IDH1 mutations were associated with prolonged overall survival. Chemotherapy and extended surgical resection were associated with improved outcome, whereas radiotherapy was not associated with overall survival and was inferior to chemotherapy alone. Conclusion: Gliomatosis cerebri among children and adolescents presents distinct histopathologic and molecular features compared to adults. However, similar associations of chemotherapy, and, when feasible, extended surgical resection, with favorable outcomes were noted among the 2 age groups.

UR - http://www.scopus.com/inward/record.url?scp=85063355621&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85063355621&partnerID=8YFLogxK

U2 - 10.1177/0883073819836551

DO - 10.1177/0883073819836551

M3 - Article

VL - 34

SP - 394

EP - 401

JO - Journal of Child Neurology

JF - Journal of Child Neurology

SN - 0883-0738

IS - 7

ER -