In the new WHO 2021 Classification of CNS Tumors the chapter “Circumscribed astrocytic gliomas, glioneuronal and neuronal tumors” encompasses several different rare tumor entities, which occur more frequently in children, adolescents, and young adults. The Task Force has reviewed the evidence of diagnostic and therapeutic interventions, which is low particularly for adult patients, and draw recommendations accordingly. Tumor diagnosis, based on WHO 2021, is primarily performed using conventional histological techniques; however, a molecular workup is important for differential diagnosis, in particular, DNA methylation profiling for the definitive classification of histologically unresolved cases. Molecular factors are increasing of prognostic and predictive importance. MRI finding are non-specific, but for some tumors are characteristic and suggestive. Gross total resection, when feasible, is the most important treatment in terms of prolonging survival and achieving long-term seizure control. Conformal radiotherapy should be considered in grade 3 and incompletely resected grade 2 tumors. In recurrent tumors reoperation and radiotherapy, including stereotactic radiotherapy, can be useful. Targeted therapies may be used in selected patients: BRAF and MEK inhibitors in pilocytic astrocytomas, pleomorphic xanthoastrocytomas, and gangliogliomas when BRAF altered, and mTOR inhibitor everolimus in subependymal giant cells astrocytomas. Sequencing to identify molecular targets is advocated for diagnostic clarification and to direct potential targeted therapies.

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Roberta Rudà, David Capper, Adam D Waldman, Johan Pallud, Giuseppe Minniti, Thomas J Kaley, Eric Bouffet, Ghazaleh Tabatabai, Eleonora Aronica, Asgeir S Jakola, Stefan M Pfister, David Schiff, Andrew B Lassman, David A Solomon, Riccardo Soffietti, Michael Weller, Matthias Preusser, Ahmed Idbaih, Patrick Y Wen, Martin J van den Bent, EANO - EURACAN - SNO Guidelines on circumscribed astrocytic gliomas, glioneuronal, and neuronal tumors, Neuro-Oncology, 2022;, noac188, https://doi.org/10.1093/neuonc/noac188