中枢神经系统孤立性纤维瘤诊治进展
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马综综

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Advances in Diagnosis and Management of Solitary Fibrous Tumor of the Central Nervous System
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1.Nan shi Hospital of Nanyang;2.华中科技大学同济医学院附属同济医院

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    中枢神经系统孤立性纤维瘤(Solitary Fibrous Tumor, SFT)是一种罕见的中枢神经系统间叶源性肿瘤,其临床表现与影像学特征常与脑膜瘤等其他颅内肿瘤相似,导致术前准确诊断较为困难。根据2021年WHO中枢神经系统肿瘤分类,SFT与血管外皮细胞瘤被归并为“SFT/HPC”这一统一疾病实体。该肿瘤的发生与NAB2-STAT6基因融合密切相关,其核蛋白表达产物STAT6已成为诊断该病的特异性分子标志物。本文系统综述了中枢神经系统SFT的流行病学特征、发病机制、临床表现、影像学及病理学表现、鉴别诊断要点以及综合治疗策略。手术全切除是治疗的基础,术后需根据WHO分级和手术切除范围等因素制定个体化辅助治疗方案。对于高级别肿瘤或未能全切的患者,辅助放疗有助于提高局部控制率;而在复发或晚期病例中,抗血管生成靶向治疗及针对成纤维细胞激活蛋白α的放射性核素治疗等新兴疗法显示出良好的应用前景。建立以神经外科为核心的多学科协作诊疗模式,对于实现SFT的精准诊疗和改善患者预后具有重要意义。

    Abstract:

    Solitary Fibrous Tumor (SFT) of the central nervous system (CNS) is a rare mesenchymal tumor. Its clinical manifestations and neuroimaging features are often nonspecific and can mimic other intracranial tumors, particularly meningiomas, making accurate preoperative diagnosis challenging. The 2021 World Health Organization (WHO) classification of CNS tumors has merged SFT and hemangiopericytoma (HPC) into a single diagnostic entity designated as "SFT/HPC". Pathogenetically, this tumor is defined by a characteristic NAB2-STAT6 gene fusion, and consequent nuclear immunoreactivity for STAT6 protein serves as a highly specific diagnostic marker.This review comprehensively examines the epidemiology, pathogenesis, clinical presentation, radiological and pathological characteristics, differential diagnosis, and management strategies for CNS SFT. Gross total resection remains the cornerstone of treatment. The decision for postoperative adjuvant therapy should be individualized based on factors such as the WHO grade and the extent of resection. Adjuvant radiotherapy significantly improves local control in patients with high-grade tumors or following subtotal resection. For recurrent or advanced disease, emerging therapies, including anti-angiogenic agents and fibroblast activation protein-alpha (FAP)-targeted radioligand therapy, show promising results.Establishing a multidisciplinary team approach, led by neurosurgery, is crucial for optimizing the precision diagnosis and treatment of SFT and for improving patient outcomes.

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  • 收稿日期:2025-10-21
  • 最后修改日期:2025-11-30
  • 录用日期:2025-12-01
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