Chapter 9 - The role of biomarkers in the diagnosis and treatment of oligodendrogliomas

Abstract

Oligodendrogliomas are rare primary brain tumors that are molecularly distinct from astrocytomas and tend to be more chemosensitive and less aggressive. The diagnosis of oligodendroglioma is now based on the presence of mutations in both isocitrate dehydrogenase (IDH) 1 or 2 genes and co-deletion of chromosome arms 1p and 19q. The optimal treatment for anaplastic oligodendrogliomas remains controversial and there is significant heterogeneity of practice within the field of neuro-oncology. Here we review the biology of oligodendrogliomas focusing on IDH mutations, 1p/19q co-deletion, and mutations in the TERT promoter. Treatment trials leading to current practices are discussed.