TY - JOUR
T1 - Rare Primary Central Nervous System Tumors in Adults
T2 - An Overview
AU - EURACAN Domain 10
AU - Franceschi, Enrico
AU - Frappaz, Didier
AU - Rudà, Roberta
AU - Hau, Peter
AU - Preusser, Matthias
AU - Houillier, Caroline
AU - Lombardi, Giuseppe
AU - Asioli, Sofia
AU - Dehais, Caroline
AU - Bielle, Franck
AU - Di Nunno, Vincenzo
AU - van den Bent, Martin
AU - Brandes, Alba A.
AU - Idbaih, Ahmed
AU - Wagemakers, Michiel
N1 - Publisher Copyright:
© Copyright © 2020 Franceschi, Frappaz, Rudà, Hau, Preusser, Houillier, Lombardi, Asioli, Dehais, Bielle, Di Nunno, van den Bent, Brandes, Idbaih and EURACAN Domain 10.
PY - 2020/6/26
Y1 - 2020/6/26
N2 - 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 Union has launched European Reference Networks (ERNs) dedicated to support advances on the clinical side of rare diseases including rare cancers. The ERN for rare solid adult tumors is termed EURACAN. Within EURACAN, Domain 10 brings together the European patient advocacy groups (ePAGs) and physicians dedicated to improving outcomes in rare primary CNS tumors and also aims at supporting research, care and teaching in the field. In this review, we discuss the relevant biological and clinical characteristics, clinical management of patients, and research directions for the following types of rare primary CNS tumors: medulloblastoma, pineal region tumors, glioneuronal and rare glial tumors, ependymal tumors, grade III meningioma and mesenchymal tumors, primary central nervous system lymphoma, germ cell tumors, spinal cord tumors and rare pituitary tumors.
AB - 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 Union has launched European Reference Networks (ERNs) dedicated to support advances on the clinical side of rare diseases including rare cancers. The ERN for rare solid adult tumors is termed EURACAN. Within EURACAN, Domain 10 brings together the European patient advocacy groups (ePAGs) and physicians dedicated to improving outcomes in rare primary CNS tumors and also aims at supporting research, care and teaching in the field. In this review, we discuss the relevant biological and clinical characteristics, clinical management of patients, and research directions for the following types of rare primary CNS tumors: medulloblastoma, pineal region tumors, glioneuronal and rare glial tumors, ependymal tumors, grade III meningioma and mesenchymal tumors, primary central nervous system lymphoma, germ cell tumors, spinal cord tumors and rare pituitary tumors.
KW - CNS lymphoma
KW - embryonal tumor of central nervous system
KW - germ cell tumors
KW - glioneural tumor
KW - medullobalstoma
KW - mesenchymal non meningothelial intracranial tumors
KW - pineal tumors
KW - pituitary tumor
UR - http://www.scopus.com/inward/record.url?scp=85087770629&partnerID=8YFLogxK
U2 - 10.3389/fonc.2020.00996
DO - 10.3389/fonc.2020.00996
M3 - Review article
AN - SCOPUS:85087770629
SN - 2234-943X
VL - 10
JO - Frontiers in Oncology
JF - Frontiers in Oncology
M1 - 996
ER -