Clinical, neuroimaging and histopathological features of gliomatosis cerebri

a systematic review based on synthesis of published individual patient data

Marios K. Georgakis, Georgios Tsivgoulis, Dimitrios Spinos, Nikolaos G. Dimitriou, Athanasios P. Kyritsis, Ulrich Herrlinger, Eleni Th Petridou

Research output: Contribution to journalReview article

2 Citations (Scopus)

Abstract

Introduction: Gliomatosis cerebri (GC) is a rare fatal widespread infiltrating CNS tumor. As consistent disease features have not been established, the tumor comprises a diagnostic challenge. Methods: We conducted a systematic literature search for published case reports and case series on patients with histologically confirmed GC. Clinical, diagnostic, neuroimaging, histopathological, and molecular data on individual or summary patient level were extracted and analyzed. Results: A total of 274 studies were identified, including 866 patients with individual-level data and 782 patients with summary data (58.9% males, mean age 43.6 years). Seizures (49.8%) were the most common presenting symptom followed by headache (35.9%), cognitive decline (32.2%), and focal motor deficits (32%). Imaging studies showed bilateral hemisphere involvement in 65%, infratentorial infiltration in 29.9% and a focal contrast-enhanced mass (type II GC) in 31.1% of cases. MRI (extensive hyperintensities in T2/FLAIR sequences) and MR spectroscopy (elevated choline, creatinine, and myoinositol levels; decreased NAA levels) showed highly consistent findings across GC patients. Low-grade and anaplastic astrocytoma were the most prevalent diagnostic categories, albeit features of any histology (astrocytic, oligodendroglial, oligoastrocytic) and grade (II–IV) were also reported. Among molecular aberrations, IDH1 mutation and MGMT promoter methylation were the most commonly reported. Increasing time elapsed from symptom onset to diagnosis comprised the only independent determinant of the extent of CNS infiltration. Conclusion: A distinct clinical, neuroimaging, histopathological, or molecular GC phenotype is not supported by current evidence. MRI and MR spectroscopy are important tools for the diagnosis of the tumor before confirmation with biopsy.

Original languageEnglish (US)
Pages (from-to)467-475
Number of pages9
JournalJournal of Neuro-Oncology
Volume140
Issue number2
DOIs
StatePublished - Nov 15 2018

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Neuroepithelial Neoplasms
Neuroimaging
Magnetic Resonance Spectroscopy
Neoplasms
Astrocytoma
Inositol
Choline
Methylation
Headache
Creatinine
Histology
Seizures
Phenotype
Biopsy
Mutation

All Science Journal Classification (ASJC) codes

  • Oncology
  • Neurology
  • Clinical Neurology
  • Cancer Research

Cite this

Clinical, neuroimaging and histopathological features of gliomatosis cerebri : a systematic review based on synthesis of published individual patient data. / Georgakis, Marios K.; Tsivgoulis, Georgios; Spinos, Dimitrios; Dimitriou, Nikolaos G.; Kyritsis, Athanasios P.; Herrlinger, Ulrich; Petridou, Eleni Th.

In: Journal of Neuro-Oncology, Vol. 140, No. 2, 15.11.2018, p. 467-475.

Research output: Contribution to journalReview article

Georgakis, Marios K. ; Tsivgoulis, Georgios ; Spinos, Dimitrios ; Dimitriou, Nikolaos G. ; Kyritsis, Athanasios P. ; Herrlinger, Ulrich ; Petridou, Eleni Th. / Clinical, neuroimaging and histopathological features of gliomatosis cerebri : a systematic review based on synthesis of published individual patient data. In: Journal of Neuro-Oncology. 2018 ; Vol. 140, No. 2. pp. 467-475.
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title = "Clinical, neuroimaging and histopathological features of gliomatosis cerebri: a systematic review based on synthesis of published individual patient data",
abstract = "Introduction: Gliomatosis cerebri (GC) is a rare fatal widespread infiltrating CNS tumor. As consistent disease features have not been established, the tumor comprises a diagnostic challenge. Methods: We conducted a systematic literature search for published case reports and case series on patients with histologically confirmed GC. Clinical, diagnostic, neuroimaging, histopathological, and molecular data on individual or summary patient level were extracted and analyzed. Results: A total of 274 studies were identified, including 866 patients with individual-level data and 782 patients with summary data (58.9{\%} males, mean age 43.6 years). Seizures (49.8{\%}) were the most common presenting symptom followed by headache (35.9{\%}), cognitive decline (32.2{\%}), and focal motor deficits (32{\%}). Imaging studies showed bilateral hemisphere involvement in 65{\%}, infratentorial infiltration in 29.9{\%} and a focal contrast-enhanced mass (type II GC) in 31.1{\%} of cases. MRI (extensive hyperintensities in T2/FLAIR sequences) and MR spectroscopy (elevated choline, creatinine, and myoinositol levels; decreased NAA levels) showed highly consistent findings across GC patients. Low-grade and anaplastic astrocytoma were the most prevalent diagnostic categories, albeit features of any histology (astrocytic, oligodendroglial, oligoastrocytic) and grade (II–IV) were also reported. Among molecular aberrations, IDH1 mutation and MGMT promoter methylation were the most commonly reported. Increasing time elapsed from symptom onset to diagnosis comprised the only independent determinant of the extent of CNS infiltration. Conclusion: A distinct clinical, neuroimaging, histopathological, or molecular GC phenotype is not supported by current evidence. MRI and MR spectroscopy are important tools for the diagnosis of the tumor before confirmation with biopsy.",
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T1 - Clinical, neuroimaging and histopathological features of gliomatosis cerebri

T2 - a systematic review based on synthesis of published individual patient data

AU - Georgakis, Marios K.

AU - Tsivgoulis, Georgios

AU - Spinos, Dimitrios

AU - Dimitriou, Nikolaos G.

AU - Kyritsis, Athanasios P.

AU - Herrlinger, Ulrich

AU - Petridou, Eleni Th

PY - 2018/11/15

Y1 - 2018/11/15

N2 - Introduction: Gliomatosis cerebri (GC) is a rare fatal widespread infiltrating CNS tumor. As consistent disease features have not been established, the tumor comprises a diagnostic challenge. Methods: We conducted a systematic literature search for published case reports and case series on patients with histologically confirmed GC. Clinical, diagnostic, neuroimaging, histopathological, and molecular data on individual or summary patient level were extracted and analyzed. Results: A total of 274 studies were identified, including 866 patients with individual-level data and 782 patients with summary data (58.9% males, mean age 43.6 years). Seizures (49.8%) were the most common presenting symptom followed by headache (35.9%), cognitive decline (32.2%), and focal motor deficits (32%). Imaging studies showed bilateral hemisphere involvement in 65%, infratentorial infiltration in 29.9% and a focal contrast-enhanced mass (type II GC) in 31.1% of cases. MRI (extensive hyperintensities in T2/FLAIR sequences) and MR spectroscopy (elevated choline, creatinine, and myoinositol levels; decreased NAA levels) showed highly consistent findings across GC patients. Low-grade and anaplastic astrocytoma were the most prevalent diagnostic categories, albeit features of any histology (astrocytic, oligodendroglial, oligoastrocytic) and grade (II–IV) were also reported. Among molecular aberrations, IDH1 mutation and MGMT promoter methylation were the most commonly reported. Increasing time elapsed from symptom onset to diagnosis comprised the only independent determinant of the extent of CNS infiltration. Conclusion: A distinct clinical, neuroimaging, histopathological, or molecular GC phenotype is not supported by current evidence. MRI and MR spectroscopy are important tools for the diagnosis of the tumor before confirmation with biopsy.

AB - Introduction: Gliomatosis cerebri (GC) is a rare fatal widespread infiltrating CNS tumor. As consistent disease features have not been established, the tumor comprises a diagnostic challenge. Methods: We conducted a systematic literature search for published case reports and case series on patients with histologically confirmed GC. Clinical, diagnostic, neuroimaging, histopathological, and molecular data on individual or summary patient level were extracted and analyzed. Results: A total of 274 studies were identified, including 866 patients with individual-level data and 782 patients with summary data (58.9% males, mean age 43.6 years). Seizures (49.8%) were the most common presenting symptom followed by headache (35.9%), cognitive decline (32.2%), and focal motor deficits (32%). Imaging studies showed bilateral hemisphere involvement in 65%, infratentorial infiltration in 29.9% and a focal contrast-enhanced mass (type II GC) in 31.1% of cases. MRI (extensive hyperintensities in T2/FLAIR sequences) and MR spectroscopy (elevated choline, creatinine, and myoinositol levels; decreased NAA levels) showed highly consistent findings across GC patients. Low-grade and anaplastic astrocytoma were the most prevalent diagnostic categories, albeit features of any histology (astrocytic, oligodendroglial, oligoastrocytic) and grade (II–IV) were also reported. Among molecular aberrations, IDH1 mutation and MGMT promoter methylation were the most commonly reported. Increasing time elapsed from symptom onset to diagnosis comprised the only independent determinant of the extent of CNS infiltration. Conclusion: A distinct clinical, neuroimaging, histopathological, or molecular GC phenotype is not supported by current evidence. MRI and MR spectroscopy are important tools for the diagnosis of the tumor before confirmation with biopsy.

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U2 - 10.1007/s11060-018-2976-4

DO - 10.1007/s11060-018-2976-4

M3 - Review article

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