Intracranial ependymomas: molecular insights and translation to treatment

Lester, Ashleigh; McDonald, Kerrie L.

doi:10.1111/bpa.12781

Cited by 25 publications

(20 citation statements)

References 104 publications

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“…Recent large-scale genomic sequencing studies have identified distinct subgroups of Supratentorial (ST)-Ependymoma, characterized by distinct methylation and gene expression patterns and the presence of characteristic gene fusion products that represent the likely tumor-initiating events and oncogenic drivers in these tumors (Lester & McDonald, 2020;Pajtler et al, 2015;Parker et al, 2014).…”

Section: Modeling Of Less Common Cns Tumorsmentioning

confidence: 99%

Leveraging the replication‐competent avian‐like sarcoma virus/tumor virus receptor‐A system for modeling human gliomas

Kanvinde

Malla

Connolly

et al. 2021

Glia

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Gliomas are the most common primary intrinsic brain tumors occurring in adults. Of all malignant gliomas, glioblastoma (GBM) is considered the deadliest tumor type due to diffuse brain invasion, immune evasion, cellular, and molecular heterogeneity, and resistance to treatments resulting in high rates of recurrence. An extensive understanding of the genomic and microenvironmental landscape of gliomas gathered over the past decade has renewed interest in pursuing novel therapeutics, including immune checkpoint inhibitors, glioma‐associated macrophage/microglia (GAMs) modulators, and others. In light of this, predictive animal models that closely recreate the conditions and findings found in human gliomas will serve an increasingly important role in identifying new, effective therapeutic strategies. Although numerous syngeneic, xenograft, and transgenic rodent models have been developed, few include the full complement of pathobiological features found in human tumors, and therefore few accurately predict bench‐to‐bedside success. This review provides an update on how genetically engineered rodent models based on the replication‐competent avian‐like sarcoma (RCAS) virus/tumor virus receptor‐A (tv‐a) system have been used to recapitulate key elements of human gliomas in an immunologically intact host microenvironment and highlights new approaches using this model system as a predictive tool for advancing translational glioma research.

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Section: Modeling Of Less Common Cns Tumorsmentioning

confidence: 99%

Leveraging the replication‐competent avian‐like sarcoma virus/tumor virus receptor‐A system for modeling human gliomas

Kanvinde

Malla

Connolly

et al. 2021

Glia

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“…When the pathway is disabled, the oncoprotein can translocate to the nucleus and interact with several transcription factors, causing increased cellular replication and reduced apoptosis. 10…”

Section: Classificationmentioning

confidence: 99%

Intracranial ependymomas: The role of advanced neuroimaging in diagnosis and management

et al. 2021

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Intracranial ependymomas represent a rare subgroup of glial tumours, showing a wide variety of imaging characteristics, often representing a challenging diagnosis for neuroradiologists. Here, we review the most recent scientific Literature on intracranial ependymomas, highlighting the most characteristic computed tomography and magnetic resonance imaging features of these neoplasms, along with epidemiologic data, recent classification aspects, clinical presentation and conventional therapeutic strategies. In addition, we report an illustrative case of an 18-year-old girl presenting with an intracranial supratentorial, anaplastic ependymoma, with the aim of contributing to the existing knowledge and comprehension of this rare tumour.

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“…Typically, they are believed to arise from the ependymal lining of the ventricles, cerebral hemispheres and central canal of the spinal cord. Intracranial ependymomas are rare primary tumors, accounting for 2.5% of all intracranial gliomas and 7% of primary central nervous system tumors diagnosed annually [ 1 , 2 ]. They account for 5–12% of brain tumors in children and 1–3% of brain tumors in adults [ 1 , 3 , 4 ].…”

Section: Introductionmentioning

confidence: 99%

“…They account for 5–12% of brain tumors in children and 1–3% of brain tumors in adults [ 1 , 3 , 4 ]. The incidence of ependymomas is estimated to be 0.43 patients per 100,000 population [ 1 , 2 ].…”

Section: Introductionmentioning

confidence: 99%

An Overview of Intracranial Ependymomas in Adults

Lombardi

Puppa

Pizzi

et al. 2021

Cancers

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Ependymomas are rare primary central nervous system tumors. They can form anywhere along the neuraxis, but in adults, these tumors predominantly occur in the spine and less frequently intracranially. Ependymal tumors represent a heterogenous group of gliomas, and the WHO 2016 classification is based essentially on a grading system, with ependymomas classified as grade I, II (classic), or III (anaplastic). In adults, surgery is the primary initial treatment, while radiotherapy is employed as an adjuvant treatment in some cases of grade II and in all cases of anaplastic ependymoma; chemotherapy is reserved for recurrent cases. In recent years, important and interesting advances in the molecular characterization of ependymomas have been made, allowing for the identification of nine molecular subgroups of ependymal tumors and moving toward subgroup-specific patients with improved risk stratification for treatment-decisions and future prospective trials. New targeted agents or immunotherapies for ependymoma patients are being explored for recurrent disease. This review summarizes recent molecular advances in the diagnosis and treatment of intracranial ependymomas including surgery, radiation therapy and systemic therapies.

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Intracranial ependymomas: molecular insights and translation to treatment

Cited by 25 publications

References 104 publications

Leveraging the replication‐competent avian‐like sarcoma virus/tumor virus receptor‐A system for modeling human gliomas

Leveraging the replication‐competent avian‐like sarcoma virus/tumor virus receptor‐A system for modeling human gliomas

Intracranial ependymomas: The role of advanced neuroimaging in diagnosis and management

An Overview of Intracranial Ependymomas in Adults

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