Rare cancer of the brain and of the spinal cord

Histological and molecular diagnosis requires expert centres. Case classifications further add complexity to the field and the development of clinical and translational research, in particular with new agents.
The revised World Health Organization (WHO) classification of brain tumours, published in 2016, marked the entry of neuro-oncology into the era of molecular precision medicine. Since WHO 2016, the case classification of primary brain tumours is not only based on pathology but also molecular biology, allowing individualization of particular entities based on both pathological and molecular features.

Close to one hundred and fifty entities have been listed in the last WHO 2021 classification.
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The most common primary brain tumours are diffuse glial tumours including astrocytomas and oligodendrogliomas from grades 2 to 4.
Other typesof rare brain tumours can be merged into a number of clinically relevant groups: (i) medulloblastoma and embryonal tumours, (ii) pineal region tumours,(iii) glioneuronal tumours and rare glial tumours, (iv) ependymal tumours, (v)grade 3 meningioma and mesenchymal non-meningothelial tumours, (vi) primary central nervous system lymphoma and haematological diseases, (vii) central nervous system germ cell tumours, (viii) spinal cord tumours and, (ix) rare pituitary tumours. These nine groups of brain tumours are ultra-rare and require collaborative and synergistic Europe-wide, multidisciplinary collaboration to achieve significant biological and clinical advances in the setting of clinical trials which would otherwise.
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As an example, in oligodendroglioma, collaborative work within the Brain Tumour Group of the European Organization for Research and Treatment of Cancer (EORTC) has allowed identification of robust diagnostic, prognostic and predictive molecular biomarkers and appropriate treatments which doubled the medianover all survival of patients. Similar efforts and results were obtained from our North American colleagues.