Abstract
Diffuse midline glioma with H3K27M mutation (H3K27M-mutant DMG) is a rare central nervous system tumor with an extremely poor prognosis. The absence of a standard treatment regimen makes achieving durable survival benefits a major therapeutic challenge. This study reports the case of a 17-year-old male diagnosed with H3K27M-mutant glioma. Following surgical resection and postoperative concurrent chemoradiotherapy, the patient received adjuvant therapy combining teniposide (VM-26) and bevacizumab, which led to significant radiological and symptomatic improvement. By examining the efficacy of this individualized comprehensive strategy—integrating surgery, chemoradiotherapy, and targeted therapy with teniposide—we assess its potential mechanisms and clinical value. This case suggests a potentially effective treatment option for patients with H3K27M-mutant DMG.