Nagai Kenichiro, Fujii Masazumi
Department of Neurosurgery, Fukushima Medical University.
No Shinkei Geka. 2023 Sep;51(5):845-857. doi: 10.11477/mf.1436204826.
Schwannomas are benign capsular tumors originating from Schwann cells. Although the majority are sporadic, they also occur within tumor predisposition syndromes, such as neurofibromatosis type 2, schwannomatosis, and Carney complex. Since the 5th edition of the World Health Organization(WHO)Classification of Tumors of the Central Nervous System was published, the description of grades has changed from Roman numerals to Arabic numerals. However, as in the 4th edition, it is still a WHO grade 1 benign tumor. There are several other subtypes of schwannomas in addition to the conventional type, and five subtypes have been specifically described in the 5th edition. "Melanocytic Schwannoma" in the 4th edition is now called "malignant melanotic nerve sheath tumor" in the 5th edition and is classified as a different tumor from schwannoma. Although the 5th edition places greater emphasis on genetic diagnoses, it is not essential for diagnosing schwannomas, and histological and clinical diagnoses remain equally crucial. Furthermore, after publication of the 5th edition in September 2022, an international consensus group renamed "neurofibromatosis type 2" as "NF2-related schwannomatosis." This article describes the shifts between the 4th to the 5th edition of the WHO Classification of Tumors of the Central Nervous System, along with additional clarifications, and offers the latest insights into treatment modalities for schwannomas and NF2.
施万细胞瘤是起源于施万细胞的良性包膜肿瘤。虽然大多数是散发性的，但也可发生于肿瘤易感综合征，如2型神经纤维瘤病、施万细胞瘤病和卡尼综合征。自世界卫生组织（WHO）中枢神经系统肿瘤分类第5版出版以来，分级的描述已从罗马数字改为阿拉伯数字。然而，与第4版一样，它仍然是WHO 1级良性肿瘤。除了传统类型外，施万细胞瘤还有其他几种亚型，第5版中特别描述了五种亚型。第4版中的“黑素细胞性施万细胞瘤”在第5版中现称为“恶性黑素性神经鞘瘤”，并被归类为与施万细胞瘤不同的肿瘤。虽然第5版更强调基因诊断，但这对施万细胞瘤的诊断并非必不可少，组织学和临床诊断仍然同样关键。此外，在2022年9月第5版出版后，一个国际共识小组将“2型神经纤维瘤病”重新命名为“NF2相关施万细胞瘤病”。本文描述了WHO中枢神经系统肿瘤分类第4版到第5版之间的变化，并给出了更多说明，同时提供了施万细胞瘤和NF2治疗方式的最新见解。
