Department of Neurosurgery, Duke University Medical Center, Durham, NC 27710, USA.
Department of Pathology, Duke University Medical Center, Durham, NC 27710, USA.
CNS Oncol. 2021 Jun 1;10(2):CNS71. doi: 10.2217/cns-2020-0030. Epub 2021 Apr 28.
Diffuse midline gliomas harboring histone H3 K27M mutations are most commonly found in the brainstem of children. This mutation confers a WHO grade IV designation and is associated with a particularly poor prognosis. Although traditionally considered to be a disease of children and young adults, a number of recent reports have described H3 K27M mutations in older adults with diffuse midline gliomas. Here, we present the unusual case of a diffuse midline glioma in the pons and cerebellum of an 83-year-old woman and review the evolving clinical literature on this entity in adults. This case underscores that it may occur even in older adults, in whom prognostic and treatment paradigms used in pediatrics may not be directly applicable.
弥漫性中线胶质瘤中存在组蛋白 H3 K27M 突变,最常见于儿童的脑干中。这种突变赋予了世界卫生组织(WHO)分级 IV ,并且与预后特别差相关。尽管传统上认为这是一种儿童和青年期的疾病,但最近有一些报告描述了在患有弥漫性中线胶质瘤的老年患者中存在 H3 K27M 突变。在这里,我们报告了一例罕见的 83 岁女性桥脑和小脑弥漫性中线胶质瘤,并回顾了关于成人这一实体的不断发展的临床文献。这个病例强调了它甚至可能发生在老年患者中,在这些患者中,儿科中使用的预后和治疗模式可能并不直接适用。