Dataset Information

NCI-CONNECT: Comprehensive Oncology Network Evaluating Rare CNS Tumors-Histone Mutated Midline Glioma Workshop Proceedings.

ABSTRACT: Histone mutations occur in approximately 4% of different cancer types. In 2012, mutations were found in the gene encoding histone variant H3.3 (H3F3A gene) in pediatric diffuse intrinsic pontine gliomas and pediatric hemispheric gliomas. Tumors with mutations in the H3F3A gene are generally characterized as histone mutated gliomas (HMGs) or diffuse midline gliomas. HMGs are a rare subtype of glial tumor that is malignant and fast growing, carrying a poor prognosis. In 2017, the Beau Biden Cancer Moonshot Program appropriated $1.7 billion toward cancer care in 10 select areas. The National Cancer Institute (NCI) was granted support to focus specifically on rare central nervous system (CNS) tumors through NCI-CONNECT. Its mission is to address the challenges and unmet needs in CNS cancer research and treatment by connecting patients, providers, researchers, and advocacy organizations to work in partnership. On September 27, 2018, NCI-CONNECT convened a workshop on histone mutated midline glioma, one of the 12 CNS cancers included in its initial portfolio. Three leaders in the field provided an overview of advances in histone mutated midline glioma research. These experts shared observations and experiences related to common scientific and clinical challenges in studying these tumors. Although the clinical focus of this workshop was on adult patients, one important objective was to start a collaborative dialogue between pediatric and adult clinicians and researchers. Meeting participants identified needs for diagnostic and treatment standards, disease biology and biological targets for this cancer, disease-specific trial designs, and developed a list of action items and future direction.

SUBMITTER: Theeler BJ

PROVIDER: S-EPMC7212875 | biostudies-literature | 2020 Jan-Dec

REPOSITORIES: biostudies-literature

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Publications

NCI-CONNECT: Comprehensive Oncology Network Evaluating Rare CNS Tumors-Histone Mutated Midline Glioma Workshop Proceedings.

Theeler Brett J BJ Dalal Yamini Y Monje Michelle M Shilatifard Ali A Suvà Mario L ML Aboud Orwa O Camphausen Kevin K Cordova Christine C Finch Elizabeth E Heiss John D JD Packer Roger J RJ Romo Carlos G CG Aldape Kenneth K Penas-Prado Marta M Armstrong Terri T Gilbert Mark R MR

Neuro-oncology advances 20200116 1

Histone mutations occur in approximately 4% of different cancer types. In 2012, mutations were found in the gene encoding histone variant H3.3 (<i>H3F3A</i> gene) in pediatric diffuse intrinsic pontine gliomas and pediatric hemispheric gliomas. Tumors with mutations in the <i>H3F3A</i> gene are generally characterized as histone mutated gliomas (HMGs) or diffuse midline gliomas. HMGs are a rare subtype of glial tumor that is malignant and fast growing, carrying a poor prognosis. In 2017, the Bea ...[more]

PMID: 32642676

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Project description:PurposePopulation-based cancer statistics, including histology-specific incidence, prevalence, and survival are essential to evaluating the total burden due to disease in a population. The National Cancer Institute's (NCI) Comprehensive Oncology Network Evaluating Rare CNS Tumors (NCI-CONNECT) was developed to better understand tumor biology and patient outcomes for 12 selected brain and other central nervous system (CNS) tumor histologies that are rare in adults to improve approaches to care and treatment. The aim of this study was to determine the incidence, prevalence, and survival of these selected rare histologies.MethodsData from the Central Brain Tumor Registry of the United States (CBTRUS) from 2000 to 2014 were used to calculate average annual age-adjusted incidence rates (AAIR) per 100,000 population overall and by sex, race, ethnicity, and age. NCI's Surveillance, Epidemiology and End Results (SEER) data were used to calculate relative survival (RS) estimates. Point prevalence for 2014 was estimated using annual age-specific incidence and survival from CBTRUS and SEER, respectively.ResultsOverall AAIR was 1.47 per 100,000 for all 12 rare histologies combined, with the highest histology-specific incidence in oligodendrogliomas (AAIR = 0.40/100,000). Overall, most histologies were more common in males, adults (age 40 + ), Whites, and non-Hispanics. Ependymomas were the most prevalent histology at 4.11 per 100,000; followed by oligodendrogliomas at 3.68 per 100,000. Relative survival at 1-, 5-, and 10-years was 82.3%, 64.0%, and 55.4%, respectively for all 12 selected brain and other CNS tumor types combined. Ependymomas had the highest RS (1-year = 94.2%, 5-year = 83.9%, 10-year = 78.6%) and gliosarcomas had the lowest relative survival rate (1-year = 42.5%, 5-year = 5.6%, 10-year = 2.9%) at all three time points.ConclusionsIncidence and prevalence of these rare brain and other CNS tumor histologies have not been previously reported. Along with survival, these data provide a statistical foundation to understand the impact of these cancers and provide important disease-specific data for the design of prospective clinical trials.

Dataset Information

NCI-CONNECT: Comprehensive Oncology Network Evaluating Rare CNS Tumors-Histone Mutated Midline Glioma Workshop Proceedings.

Publications

NCI-CONNECT: Comprehensive Oncology Network Evaluating Rare CNS Tumors-Histone Mutated Midline Glioma Workshop Proceedings.

Similar Datasets

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