根据2021年世界卫生组织（WHO）中枢神经系统肿瘤分类，婴儿型半球胶质瘤属于儿科类型的弥漫性高级别胶质瘤。其特征为酪氨酸激酶基因重排（NTRK1/2/3，ALK，ROS1，MET）。本研究的目的是描述这类肿瘤的临床、组织病理和分子特征，并对文献进行回顾。本回顾性研究系2020年至2022年期间在昂热大学医院诊断的四例婴儿型半球胶质瘤。基于形态学和免疫组织化学，怀疑诊断，并通过分子生物学技术得到确认。最常见的临床表现是颅内压增高。影像学显示大脑半球肿瘤且对比增强。显微镜检查显示弥漫性星形细胞瘤具有高级别特征，有时伴有神经元或假表皮样分化。通过鉴定涉及酪氨酸激酶基因的基因融合，可以确诊婴儿型半球胶质瘤。婴儿型半球胶质瘤罕见，常见于非常幼小儿童，并表现为大脑半球肿瘤。处理婴儿高级别胶质瘤时，应系统地寻找酪氨酸激酶基因融合。重要的是，这些基因融合是治疗靶点。靶向治疗对患者存活率的影响应在未来的前瞻性研究中进行评估。 版权所有 © 2023 Elsevier Masson SAS。保留所有权利。

Infant-type hemispheric gliomas belong to pediatric-type diffuse high-grade gliomas according to the 2021 WHO classification of central nervous system tumors. They are characterized by tyrosine kinase gene rearrangements (NTRK1/2/3, ALK, ROS1, MET). The aim of the study was to describe the clinical, histopathologic, and molecular characteristics of such tumors, and to provide a review of the literature.This retrospective series comprises four cases of infant-type hemispheric glioma diagnosed at Angers University Hospital between 2020 and 2022. The diagnosis was suspected based on morphology and immunohistochemistry and was confirmed by molecular biology techniques.The most common clinical sign was raised intracranial pressure. Imaging showed a large cerebral hemispheric tumor with contrast enhancement. Microscopic examination revealed diffuse astrocytoma with high-grade features, sometimes with neuronal or pseudo-ependymal differentiation. Identification of a gene fusion involving a tyrosine kinase gene allowed to make a definitive diagnosis of infant-type hemispheric glioma.Infant-type hemispheric gliomas are rare and present as large cerebral hemispheric tumors in very young children. Searching for a tyrosine kinase gene fusion should be systematic when dealing with a high-grade glioma in an infant. Importantly, these gene fusions are therapeutic targets. The impact of targeted therapies on patient survival should be evaluated in future prospective studies.Copyright © 2023 Elsevier Masson SAS. All rights reserved.