Preprint Review Version 1 Preserved in Portico This version is not peer-reviewed

The Molecular, Neurological, and Clinical Features of Diffuse Intrinsic Pontine Glioma

Version 1 : Received: 7 November 2022 / Approved: 8 November 2022 / Online: 8 November 2022 (02:57:43 CET)

How to cite: Denniston, K.; Dickson, A.; Fitzsimmons, A.; Verbeke-O'Boyle, G.; Piper, B. The Molecular, Neurological, and Clinical Features of Diffuse Intrinsic Pontine Glioma. Preprints 2022, 2022110140. https://doi.org/10.20944/preprints202211.0140.v1 Denniston, K.; Dickson, A.; Fitzsimmons, A.; Verbeke-O'Boyle, G.; Piper, B. The Molecular, Neurological, and Clinical Features of Diffuse Intrinsic Pontine Glioma. Preprints 2022, 2022110140. https://doi.org/10.20944/preprints202211.0140.v1

Abstract

Diffuse intrinsic pontine glioma (DIPG) is recognized as a pediatric brainstem cancer with a 0% survival rate. On a molecular basis, DIPG commonly results from mutations in histone H3, specifically a mutation in the H3K27M gene, that promotes tumorigenesis and results in presentation of this fatal brainstem tumor. DIPG is challenging to treat, as surgical intervention is inefficacious due to the location where the glioma resides. To date, traditional treatments such as radiation, chemotherapy, and immunotherapy have not increased survival rates and have only been successful at relieving symptoms. Future therapeutic approaches such as proton beam radiation, Chimeric Antigen Receptor T Cell (CAR-T) immunotherapy, and alternative epigenetic pharmaceuticals are under investigation for potential benefits. Various clinical trials have also explored these treatment procedures to discover potential increases in survival rates in both animal and human studies. In this review, we will evaluate the pathology and molecular characteristics of DIPG, the current and future approaches to DIPG treatment; and, lastly, we will discuss clinical trials that have been completed to develop successful treatment options.

Keywords

Diffuse Intrinsic PontineDiffuse Intrinsic Pontine Glioma; DIPG; pediatric tumors; cancer; CAR-T; pons; histone mutation

Subject

Medicine and Pharmacology, Neuroscience and Neurology

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