1. Rare Primary Central Nervous System Tumors in Adults: An Overview
- Author
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Enrico Franceschi, Didier Frappaz, Roberta Rudà, Peter Hau, Matthias Preusser, Caroline Houillier, Giuseppe Lombardi, Sofia Asioli, Caroline Dehais, Franck Bielle, Vincenzo Di Nunno, Martin van den Bent, Alba A. Brandes, Ahmed Idbaih, EURACAN Domain 10, Paul Clement Radek, Lakomý Nicolai El-Hindy, Jean-Yves Delattre, Ville Vuorinen, Silvia Scoccianti, Riccardo SoffiettiLucia Monti, Andrea Pace, Gaetano Finocchiaro, Arimantas TamasauskasMark ter Laan, Anja Gijtenbeek, Michiel Wagemakers, David NoskeUroš Smrdel, Puneet Plaha, Naomi Fersht, Istituti di Ricovero e Cura a Carattere Scientifico (IRCCS), Institut d'hématologie et d'oncologie pédiatrique [CHU - HCL] (IHOPe), Hospices Civils de Lyon (HCL), Centre Léon Bérard [Lyon], University of Turin, University Hospital Regensburg, Medizinische Universität Wien = Medical University of Vienna, Institut du Cerveau et de la Moëlle Epinière = Brain and Spine Institute (ICM), Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Pitié-Salpêtrière [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS), Sorbonne Université - Département de neurologie 2 - Mazarin, Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Pitié-Salpêtrière [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service de Neuropathologie [CHU Pitié Salpêtrière], CHU Pitié-Salpêtrière [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Sorbonne Université - Faculté de Médecine (SU FM), Sorbonne Université (SU), Erasmus University Medical Center [Rotterdam] (Erasmus MC), Neurology, Gestionnaire, Hal Sorbonne Université, Università degli studi di Torino = University of Turin (UNITO), Institut du Cerveau = Paris Brain Institute (ICM), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS), Service d'Onco-neurologie = Département de neurologie 2 [CHU Pitié Salpêtrière], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Franceschi E., Frappaz D., Ruda R., Hau P., Preusser M., Houillier C., Lombardi G., Asioli S., Dehais C., Bielle F., Di Nunno V., van den Bent M., Brandes A.A., and Idbaih A.
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0301 basic medicine ,Oncology ,Ependymoma ,Cancer Research ,medicine.medical_specialty ,glioneural tumor ,[SDV.NEU.NB]Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC]/Neurobiology ,[SDV.CAN]Life Sciences [q-bio]/Cancer ,Review ,pituitary tumor ,lcsh:RC254-282 ,03 medical and health sciences ,0302 clinical medicine ,[SDV.CAN] Life Sciences [q-bio]/Cancer ,Internal medicine ,pineal tumors ,medicine ,media_common.cataloged_instance ,germ cell tumors ,European union ,mesenchymal non meningothelial intracranial tumor ,media_common ,Medulloblastoma ,Grade III Meningioma ,business.industry ,CNS lymphoma ,mesenchymal non meningothelial intracranial tumors ,Pituitary tumors ,[SDV.NEU.NB] Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC]/Neurobiology ,Primary central nervous system lymphoma ,germ cell tumor ,lcsh:Neoplasms. Tumors. Oncology. Including cancer and carcinogens ,medicine.disease ,3. Good health ,embryonal tumor of central nervous system ,030104 developmental biology ,030220 oncology & carcinogenesis ,medullobalstoma ,Germ cell tumors ,business ,pineal tumor ,Rare disease - Abstract
International audience; 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.
- Published
- 2020
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