Franceschi, Enrico and Frappaz, Didier and Ruda, Roberta and Hau, Peter and Preusser, Matthias and Houillier, Caroline and Lombardi, Giuseppe and Asioli, Sofia and Dehais, Caroline and Bielle, Franck and Di Nunno, Vincenzo and van den Bent, Martin and Brandes, Alba A. and Idbaih, Ahmed (2020) Rare Primary Central Nervous System Tumors in Adults: An Overview. FRONTIERS IN ONCOLOGY, 10: 996. ISSN 2234-943X
Full text not available from this repository. (Request a copy)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 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.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | GERM-CELL TUMORS; SOLITARY FIBROUS TUMOR; PRIMARY CNS LYMPHOMA; INTERNATIONAL EXTRANODAL LYMPHOMA; CRANIOSPINAL RADIATION-THERAPY; WHOLE-BRAIN RADIOTHERAPY; HIGH-DOSE METHOTREXATE; PHASE-II; ADJUVANT CHEMOTHERAPY; EPENDYMAL TUMORS; pineal tumors; mesenchymal non meningothelial intracranial tumors; CNS lymphoma; germ cell tumors; pituitary tumor; glioneural tumor; embryonal tumor of central nervous system; medullobalstoma |
| Subjects: | 600 Technology > 610 Medical sciences Medicine |
| Divisions: | Medicine > Lehrstuhl für Neurologie |
| Depositing User: | Petra Gürster |
| Date Deposited: | 13 Apr 2021 07:35 |
| Last Modified: | 13 Apr 2021 07:35 |
| URI: | https://pred.uni-regensburg.de/id/eprint/44346 |
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