Glioneuronal tumors: clinicopathological findings and treatment options

Abstract

Glioneuronal tumors are very rare CNS neoplasms that demonstrate neuronal differentiation, composed of mixed glial and neuronal cells. The majority of these lesions are low grade and their correct classification is crucial in order to avoid misidentification as ‘ordinary’ gliomas and prevent inappropriate aggressive treatment; nevertheless, precise diagnosis is a challenge due to phenotypic overlap across different histologic subtype. Surgery is the standard of therapeutic approach; literature concerning the benefit of adjuvant treatments is inconclusive and a globally accepted treatment of recurrence does not exist. Targetable mutations in the genes BRAF and FGFR1/2 are recurrently found in these tumors and could take a promising role in future treatment management.