Department of Neurosurgery, University Clinical Hospital of Valladolid, Valladolid, Spain.
Department of Pathology, University Clinical Hospital of Valladolid, Valladolid, Spain.
Neurocirugia (Astur : Engl Ed). 2022 Nov-Dec;33(6):371-376. doi: 10.1016/j.neucie.2021.07.004.
Solitary fibrous tumors (SFTs) are neoplasms that grow from mesenchymal fusiform cells. In the central nervous system, meninges are the common origin of these neoplasms. Although literature reports mostly SFT as benign neoplasm, malignancy data have been described in recurrences or metastatic lesions. Definitive diagnosis includes immunohistochemical profiles assessing cellular positivity for CD34, vimentin, CD99 and Bcl-2. Recent studies have demonstrated NAB2-STAT6 gene fusion as a distinct molecular feature of SFT with overexpression of the fusion protein NAB2-STAT6 in nuclei of these cells. Since several years, pathologists have grouped SFT and hemangiopericytomas (HPC) as different phenotypes of the same entity although both neoplasms do not share numerous features. This article, based on a case of a recurrent malignant SFT, aims to emphasize differences in the SFT/HPC spectrum due to the diagnostic, therapeutic and prognostic implications.
孤立性纤维瘤（SFT）是一种起源于间叶梭形细胞的肿瘤。在中枢神经系统中，脑膜是这些肿瘤的常见起源。尽管文献中大多将 SFT 报告为良性肿瘤，但在复发或转移病变中已经描述了恶性数据。明确的诊断包括免疫组织化学分析，评估细胞对 CD34、波形蛋白、CD99 和 Bcl-2 的阳性表达。最近的研究表明，NAB2-STAT6 基因融合是 SFT 的一个独特分子特征，这些细胞的核内过度表达融合蛋白 NAB2-STAT6。几年来，病理学家将 SFT 和血管外皮细胞瘤（HPC）归类为同一实体的不同表型，尽管这两种肿瘤没有许多共同特征。本文基于一例复发性恶性 SFT，旨在强调 SFT/HPC 谱中的差异，因为这涉及到诊断、治疗和预后的影响。
