Diffuse midline glioma treated with epigenetic agent-based immunotherapy
Linkai Jing, Zhihong Qian, Qiang Gao, Rui Sun, Zili Zhen, Guihuai Wang, Xuejun Yang, Haitao Li, Tiannan Guo, Wei Zhang
Abstract
Diffuse midline glioma, H3K27-altered (DMG), is a pediatric-type high-grade diffuse glioma that preferentially localizes to the brainstem or pons, thalamus, and spinal cord. Surgical resection is challenging and biopsy is often performed in most cases. To date, no conventional, targeted or immune therapy has been convincingly shown to improve patients’ overall survival (OS). Effective treatment guideline is absent and remains to be established. Prognosis of DMG patients is poor and 2-year survival rate is <10%. Noteworthily, DMG arising from spinal cord with leptomeningeal metastasis (LM) is rarely reported. Historically, LM of high-grade gliomas have largely been excluded from clinical trials owing to a particularly poor prognosis, with a median OS of 1.6–3.8 months. 1