Brainstem glioma: a review

Brainstem gliomas (BGs) are a heterogenous group of gliomas that occur predominately in children. They can be separated into groups on the basis of anatomy and clinical behavior: diffuse intrinsic pontine glioma (DIPG), exophytic medullary glioma, and tectal glioma. DIPG is the commonest BG. Median age at onset is 6.5 years and median survival is less than 1 year. Adults with DIPG survive longer, suggesting a less aggressive and biologically different tumor from that in children. Patients present with cranial nerve dysfunction, long tract signs, or ataxia, either in isolation or in combination. Magnetic resonance imaging shows an infiltrative lesion occupying most of the pons and contrast enhancement is usually not prominent. Standard treatment is fractionated radiotherapy. Platelet-derived growth factor receptor alpha and epidermal growth factor receptor mutations have been identified. Inhibitors of these growth factor receptors are being evaluated in clinical trials. Exophytic medullary and tectal gliomas are relatively indolent tumors that can often be followed closely without treatment.