Rare Primary Central Nervous System Tumors in Adults: An Overview

Frontiers in Oncology
Enrico FranceschiEURACAN Domain 10

Abstract

Overall, tumors of primary central nervous system (CNS) are quite common in adults with an incidence rate close to 30 new cases/100,000 inhabitants per year. Significant clinical and biological advances have been accomplished in the most common adult primary CNS tumors (i.e., diffuse gliomas). However, most CNS tumor subtypes are rare with an incidence rate below the threshold defining rare disease of 6.0 new cases/100,000 inhabitants per year. Close to 150 entities of primary CNS tumors have now been identified by the novel integrated histomolecular classification published by the World Health Organization (WHO) and its updates by the c-IMPACT NOW consortium (the Consortium to Inform Molecular and Practical Approaches to CNS Tumor Taxonomy). While these entities can be better classified into smaller groups either by their histomolecular features and/or by their location, assessing their treatment by clinical trials and improving the survival of patients remain challenging. Despite these tumors are rare, research, and advances remain slower compared to diffuse gliomas for instance. In some cases (i.e., ependymoma, medulloblastoma) the understanding is high because single or few driver mutations have been defined. The European U...Continue Reading

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Methods Mentioned

BETA
biopsies
surgical
biopsy
methylation profiling
exome sequencing
surgical resection
flow

Clinical Trials Mentioned

NCT01356290
NCT00918320
NCT03838042
NCT03130959
NCT02601950
NCT02601937

Software Mentioned

EURACAN

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