Rare Primary Central Nervous System Tumors in Adults: An Overview
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). Howev...
| Main Authors: | , , , , , , , , , , , , , , , , , , , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2020-06-01
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| Series: | Frontiers in Oncology |
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| Online Access: | https://www.frontiersin.org/article/10.3389/fonc.2020.00996/full |
| _version_ | 1828929527376510976 |
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| author | 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 |
| author_facet | 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 |
| author_sort | Enrico Franceschi |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-14T00:20:23Z |
| format | Article |
| id | doaj.art-3617bb6cee8e40aa8f575d602ec52f33 |
| institution | Directory Open Access Journal |
| issn | 2234-943X |
| language | English |
| last_indexed | 2024-12-14T00:20:23Z |
| publishDate | 2020-06-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Oncology |
| spelling | doaj.art-3617bb6cee8e40aa8f575d602ec52f332022-12-21T23:25:16ZengFrontiers Media S.A.Frontiers in Oncology2234-943X2020-06-011010.3389/fonc.2020.00996536977Rare Primary Central Nervous System Tumors in Adults: An OverviewEnrico Franceschi0Didier Frappaz1Roberta Rudà2Peter Hau3Matthias Preusser4Caroline Houillier5Giuseppe Lombardi6Sofia Asioli7Caroline Dehais8Franck Bielle9Vincenzo Di Nunno10Martin van den Bent11Alba A. Brandes12Ahmed Idbaih13EURACAN Domain 10Paul Clement RadekLakomý Nicolai El-HindyJean-Yves DelattreVille VuorinenSilvia ScocciantiRiccardo SoffiettiLucia MontiAndrea PaceGaetano FinocchiaroArimantas TamasauskasMark ter LaanAnja GijtenbeekMichiel WagemakersDavid NoskeUroš SmrdelPuneet PlahaNaomi FershtDepartment of Medical Oncology, Azienda USL/IRCCS Institute of Neurological Sciences, Bologna, ItalyDepartment of Neuro-Oncology and Institut d'Hématologie et d'Oncologie Pédiatrique, Centre Léon Bérard, Lyon, FranceDepartment of Neuro-Oncology, City of Health and Science and University of Turin, Turin, ItalyWilhelm Sander NeuroOncology-Unit, Department of Neurology, University Hospital Regensburg, Regensburg, GermanyDivision of Oncology, Department of Medicine I, Medical University of Vienna, Vienna, AustriaSorbonne Université, IHU, ICM, Service de Neurologie 2-Mazarin, Groupe Hospitalier Pitié-Salpêtrière, Paris, FranceDepartment of Oncology, Veneto Institute of Oncology-IRCCS, Padua, ItalySection of Anatomic Pathology “M. Malpighi”, Department of Biomedical and Neuromotor Sciences, Bellaria Hospital, Bologna, ItalySorbonne Université, Inserm, CNRS, UMR S 1127, Institut du Cerveau et de la Moelle épinière, ICM, AP-HP, Hôpitaux Universitaires La Pitié Salpêtrière - Charles Foix, Service de Neurologie 2-Mazarin, Paris, France0Department of Neuropathology, Hôpitaux Universitaires La Pitié Salpêtrière - Charles Foix, AP-HP, Sorbonne Université, SIRIC Curamus, Paris, FranceDepartment of Medical Oncology, Azienda USL/IRCCS Institute of Neurological Sciences, Bologna, Italy1The Brain Tumor Center at Erasmus MC Cancer Institute, Rotterdam, NetherlandsDepartment of Medical Oncology, Azienda USL/IRCCS Institute of Neurological Sciences, Bologna, ItalySorbonne Université, Inserm, CNRS, UMR S 1127, Institut du Cerveau et de la Moelle épinière, ICM, AP-HP, Hôpitaux Universitaires La Pitié Salpêtrière - Charles Foix, Service de Neurologie 2-Mazarin, Paris, FranceOverall, 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.https://www.frontiersin.org/article/10.3389/fonc.2020.00996/fullpineal tumorsmesenchymal non meningothelial intracranial tumorsCNS lymphomagerm cell tumorspituitary tumorglioneural tumor |
| spellingShingle | 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 Rare Primary Central Nervous System Tumors in Adults: An Overview Frontiers in Oncology pineal tumors mesenchymal non meningothelial intracranial tumors CNS lymphoma germ cell tumors pituitary tumor glioneural tumor |
| title | Rare Primary Central Nervous System Tumors in Adults: An Overview |
| title_full | Rare Primary Central Nervous System Tumors in Adults: An Overview |
| title_fullStr | Rare Primary Central Nervous System Tumors in Adults: An Overview |
| title_full_unstemmed | Rare Primary Central Nervous System Tumors in Adults: An Overview |
| title_short | Rare Primary Central Nervous System Tumors in Adults: An Overview |
| title_sort | rare primary central nervous system tumors in adults an overview |
| topic | pineal tumors mesenchymal non meningothelial intracranial tumors CNS lymphoma germ cell tumors pituitary tumor glioneural tumor |
| url | https://www.frontiersin.org/article/10.3389/fonc.2020.00996/full |
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