This review summarizes the various immunotherapy-based treatments for DMG as well as their specific limitations. Given that DMGs are not amenable to surgical resection, have only limited response to radiation, and are refractory to traditional chemotherapy, immunotherapy has emerged as a promising alternative treatment modality. Continued development of novel, efficacious therapeutic options for DMGs remains a critically important area of active investigation. Research into the underlying tumor biology and numerous clinical trials have done little to change the invariably poor prognosis. Median overall survival is less than 12 months with a 90% mortality rate at 2 years from diagnosis. Diffuse midline gliomas (DMG) are a highly aggressive and universally fatal subgroup of pediatric tumors responsible for the majority of childhood brain tumor deaths.
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