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

doi:10.1007/s11060-018-2976-4

Cited by 17 publications

(23 citation statements)

References 27 publications

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“…MRI is the best imaging modality to demonstrate GC due to its greater contrast resolution and ability to more conspicuously depict the specific degree of anatomic involvement. GC typically appears iso- to hypointense on T1-weighted imaging (T1WI), hyperintense on FLAIR and T2WI, and results in expansion of involved structures with absent or minimal enhancement [9] . Areas of enhancement are suspicious for either foci of high-grade glioma or malignant progression when seen on follow-up studies.…”

Section: Discussion and Literature Reviewmentioning

confidence: 99%

“…Areas of enhancement are suspicious for either foci of high-grade glioma or malignant progression when seen on follow-up studies. Bilateral hemispheric involvement is seen in 65% of patients, infratentorial infiltration in 30% of patients, and corpus callosum involvement is found in roughly half of patients [9] .…”

Section: Discussion and Literature Reviewmentioning

confidence: 99%

“…Treatment options for GC are poor with no standard trial-based recommendation for the initiation of therapy. Many institutions treat patients the same as a high-grade glioma patient with upfront radiation or chemo-radiation [9] . However, radiation therapy has shown no overall survival benefit on several trials [3 , 21] .…”

Section: Discussion and Literature Reviewmentioning

confidence: 99%

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Gliomatosis cerebri mimicking diffuse demyelinating disease: Case Report

Mingomataj

Soleiman

Sajan

et al. 2020

Radiology Case Reports

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Gliomatosis Cerebri (GC) is a rareand rapidly progressive pattern of growth of diffusely infiltrating gliomas with limited treatment options. Imaging findings are usually nonspecific and can mimic other neurologic disorders, including demyelination, encephalitis, and multicentric/multifocal glioma. In this report, we describe a case of a 53-year-old female who presented with left hemiparesis, global headache, and gait ataxia with imaging features initially thought to represent demyelinating disease. A combination of conventional and advanced imaging findings with brain biopsy was utilized to make the diagnosis of GC. In patients with widespread abnormalities on brain imaging, GC should strongly be considered when cortical expansion, involvement of the septum pellucidum and elevated myoinositol levels are observed and the clinical and laboratory findings are atypical for demyelination or infection. Considering GC in such cases can facilitate early biopsy with prompt diagnosis and avoid delay in appropriate treatment.

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Section: Discussion and Literature Reviewmentioning

confidence: 99%

Section: Discussion and Literature Reviewmentioning

confidence: 99%

Section: Discussion and Literature Reviewmentioning

confidence: 99%

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Gliomatosis cerebri mimicking diffuse demyelinating disease: Case Report

Mingomataj

Soleiman

Sajan

et al. 2020

Radiology Case Reports

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“…Glioblastoma (GBM), used to refer to Grade-IV astrocytoma, is the most common, aggressive, and malignant form of primary brain tumors in adults. GBM patients typically present a spectrum of generalized or focal neurologic symptoms including headaches, partial or generalized seizures, unilateral or bilateral paresis, hemiplegia, ataxia, visual defects, cognitive impairment, and personality changes (Iacob and Dinca, 2009; Burks et al, 2016; Georgakis et al, 2018). These symptoms persist throughout the course of the disease and worsen following surgical resection, severely limiting day-to-day functions, impacting patients’ quality of life, and even influencing survival outcomes (Martinez et al, 2008; Iacob and Dinca, 2009; Georgakis et al, 2018; Lee et al, 2018).…”

Section: Introductionmentioning

confidence: 99%

An Inflammatory Landscape for Preoperative Neurologic Deficits in Glioblastoma

et al. 2019

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Introduction: Patients with glioblastoma (GBM), one of the most aggressive forms of primary brain tumors, exhibit a wide range of neurologic signs, ranging from headaches to neurologic deficits and cognitive impairment, at first clinical presentation. While such variability is attributed to inter-individual differences in increased intracranial pressure, tumor infiltration, and vascular compromise, a direct association with disease stage, tumor size and location, edema, and necrotic cell death has yet to be established. The lack of specificity of neurologic symptoms often confounds the diagnosis of GBM. It also limits clinicians’ ability to elect treatment regimens that not only prolong survival but also promote symptom management and high quality of life. Methods: To decipher the heterogeneous presentation of neurologic symptoms in GBM, we investigated differences in the molecular makeup of tumors from patients with and without preoperative neurologic deficits. We used the Ivy GAP (Ivy Glioblastoma Atlas Project) database to integrate RNA sequencing data from histologically defined GBM tumor compartments and neurologic examination records for 41 patients. We investigated the association of neurologic deficits with various tumor and patient attributes. We then performed differential gene expression and co-expression network analysis to identify a transcriptional signature specific to neurologic deficits in GBM. Using functional enrichment analysis, we finally provided a comprehensive and detailed characterization of involved pathways and gene interactions. Results: An exploratory investigation of the association of tumor and patient variables with the early development of neurologic deficits in GBM revealed a lack of robust and consistent clinicopathologic prognostic factors. We detected significant differences in the expression of 728 genes (FDR-adjusted p -value ≤ 0.05 and relative fold-change ≥ 1.5), unique to the cellular tumor (CT) anatomical compartment, between neurologic deficit groups. Upregulated differentially expressed genes in CT were enriched for mesenchymal subtype-predictive genes. Applying a systems approach, we then identified co-expressed gene sets that correlated with neurological deficit manifestation (FDR-adjusted p -value < 0.1). Collectively, these findings uncovered significantly enriched immune activation, oxidative stress response, and cytokine-mediated proinflammatory processes. Conclusion: Our study posits that inflammatory processes, as well as a mesenchymal tumor subtype, are implicated in the pathophysiology of preoperative neurologic deficits in GBM.

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“…Recently, we gathered available evidence for gliomatosis cerebri by performing a systematic review and an individual-patient data meta-analysis on 866 gliomatosis cerebri patients that have been described in biomedical literature. 11,12 Using this approach, it was possible for the first time to study to such an extent the diagnostic features of the disease, its prognostic factors, and the impact of treatment on outcome. Given the well-established differences in the molecular basis and in the epidemiology of brain tumors in children, as compared to the brain tumors later in life, 13,14 in this study, we aimed to elaborate data from the abovementioned data set 11,12 to describe the diagnostic and prognostic features of gliomatosis cerebri among pediatric patients and explore whether they differ from adult gliomatosis cerebri tumors.…”

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confidence: 99%

Gliomatosis Cerebri Among Children and Adolescents: An Individual-Patient Data Meta-analysis of 182 Patients

et al. 2019

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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.

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Clinical, neuroimaging and histopathological features of gliomatosis cerebri: a systematic review based on synthesis of published individual patient data

Abstract: 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.

Cited by 17 publications

References 27 publications

Gliomatosis cerebri mimicking diffuse demyelinating disease: Case Report

Gliomatosis cerebri mimicking diffuse demyelinating disease: Case Report

An Inflammatory Landscape for Preoperative Neurologic Deficits in Glioblastoma

Gliomatosis Cerebri Among Children and Adolescents: An Individual-Patient Data Meta-analysis of 182 Patients

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