• Apisara Chanchotisatien, Jiawei Pan, Zunguo Du, Tianming Qiu, Jintai Yu, and Shuguang Chu.
• Shanghai Medical College, Fudan University, Shanghai, China.
• World Neurosurg. 2019 Jul 1; 127: 266-268.

BackgroundThe 2016 World Health Organization Classification of Tumours of the Central Nervous System was revised to include a new diagnostic entity, diffuse midline glioma, H3 K27M-mutant (DMG-K27M), a highly aggressive tumor with a mean survival time of 1 year after diagnosis. DMG-K27M is classified as a World Health Organization grade IV tumor regardless of histopathologic features, and there is currently no effective treatment for it despite ongoing research.Case DescriptionWe present a case of a 39-year-old man with a slow-growing thalamic glioma with histone H3 lysine 27-to-methionine mutation. This patient received surgical intervention 3 years after the initial discovery of the tumor. Because the patient did not receive any preoperative treatment for DMG-K27M, this case reflects 1 possible natural course of progression for this type of malignancy.ConclusionsThere are currently no effective therapeutic options for treatment of DMG-K27M. The slow tumor growth and prolonged survival time (≥ 3 years) in the absence of intervention in this case serve as a reminder that much is still not known about histone H3 lysine 27-to-methionine mutation and how it impacts the pathophysiology, diagnosis, treatment, and prognosis of the disease. Careful evaluation is warranted to determine if early intervention is the best approach when treating DMG-K27M.Copyright © 2019 Elsevier Inc. All rights reserved.

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