Immune Microenvironment and Immunotherapies for Diffuse Intrinsic Pontine Glioma

SIMPLE SUMMARY: Diffuse intrinsic pontine glioma (DIPG) is a malignant primary glial tumor that occurs in all age groups but predominates in children and is estimated to account for approximately 10–15% of pediatric brain tumors. The median age at diagnosis was 6–7 years, and the median survival of children is less than 12 months. At present, there is no effective treatment method for DIPG in the clinic. The continuous progress in immunotherapy has brought new prospects for the treatment of DIPG. In this review, we summarize the knowledge about the immune profile in DIPG and existing clinical trial results of DIPG, hoping to clarify the development of novel immunotherapies for DIPG treatment. ABSTRACT: Diffuse intrinsic pontine glioma (DIPG) is a primary glial glioma that occurs in all age groups but predominates in children and is the main cause of solid tumor-related childhood mortality. Due to its rapid progression, the inability to operate and insensitivity to most chemotherapies, there is a lack of effective treatment methods in clinical practice for DIPG patients. The prognosis of DIPG patients is extremely poor, with a median survival time of no more than 12 months. In recent years, there have been continuous breakthroughs for immunotherapies in various hematological tumors and malignant solid tumors with extremely poor prognoses, which provides new insights into tumors without effective treatment strategies. Meanwhile, with the gradual development of stereotactic biopsy techniques, it is gradually becoming easier and safer to obtain live DIPG tissue, and the understanding of the immune properties of DIPG has also increased. On this basis, a series of immunotherapy studies of DIPG are under way, some of which have shown encouraging results. Herein, we review the current understanding of the immune characteristics of DIPG and critically reveal the limitations of current immune research, as well as the opportunities and challenges for immunological therapies in DIPG, hoping to clarify the development of novel immunotherapies for DIPG treatment.