Department of Neuropathology, Sainte-Anne Hospital, 1, rue Cabanis, 75014, Paris, France.
Inserm, UMR 1266, IMA-Brain, Institut de Psychiatrie et Neurosciences de Paris, Paris, France.
Acta Neuropathol Commun. 2023 Feb 3;11(1):22. doi: 10.1186/s40478-023-01522-z.
The fifth edition of the World Health Organization Classification of Tumors of the Central Nervous System (CNS) now includes mesenchymal tumors that occur uniquely or frequently in the CNS. Moreover, this version has aligned the terminology of mesenchymal tumors with their soft tissue counterparts. New tumor types have been added, such as the "intracranial mesenchymal tumor, FET-CREB fusion-positive", the "CIC-rearranged sarcoma", and the "Primary intracranial sarcoma, DICER1-mutant". Other entities (such as rhabdomyosarcoma) have remained in the current WHO classification because these tumor types may present specificities in the CNS as compared to their soft tissue counterparts. Based on an extensive literature review, herein, we will discuss these newly recognized entities in terms of clinical observation, radiology, histopathology, genetics and outcome, and consider strategies for an accurate diagnosis. In light of this literature analysis, we will also introduce some potentially novel tumor types.
世界卫生组织第五版中枢神经系统（CNS）肿瘤分类现在包括仅或经常发生在 CNS 的间叶肿瘤。此外，该版本已将间叶肿瘤的术语与其软组织对应物对齐。已经添加了新的肿瘤类型，例如“颅内间叶肿瘤，FET-CREB 融合阳性”、“CIC 重排肉瘤”和“原发性颅内肉瘤，DICER1 突变型”。其他实体（如横纹肌肉瘤）仍保留在当前的世卫组织分类中，因为与软组织对应物相比，这些肿瘤类型在 CNS 中可能具有特异性。基于广泛的文献回顾，本文将根据临床观察、影像学、组织病理学、遗传学和结果讨论这些新认可的实体，并考虑准确诊断的策略。根据这项文献分析，我们还将介绍一些潜在的新型肿瘤类型。
