中枢神经系统孤立性纤维瘤诊治进展

Advances in the diagnosis and management of solitary fibrous tumor of the central nervous system

摘要:

中枢神经系统孤立性纤维瘤（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NGFI-A结合蛋白2（NAB2）-信号转导及转录激活蛋白6（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。该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 with similar clinical manifestations and neuroimaging features as other intracranial tumors， especially meningiomas， leading to the difficulty in accurate preoperative diagnosis. Based on the 2021 World Health Organization （WHO） classification of CNS tumors， SFT and hemangiopericytoma （HPC） are categorized into the entity designated as “SFT/HPC”. The onset of this tumor is closely associated with NAB2-STAT6 gene fusion， and the nuclear protein expression product， STAT6， has become a highly specific diagnostic marker. This article systematically reviews the epidemiology， pathogenesis， clinical manifestation， radiological and pathological characteristics， key points for differential diagnosis， and comprehensive management strategies for CNS SFT. Gross total resection remains the cornerstone of treatment， and individualized adjuvant treatment regimens should be formulated after surgery based on the factors such as WHO grade and the extent of resection. For patients with high-grade tumor and those who are not eligible for total resection， adjuvant radiotherapy can help to improve local control rate， while for patients with recurrence or an advanced stage， emerging therapy such as anti-angiogenic targeted therapy and radionuclide therapy targeting fibroblast activation protein-alpha have shown good application prospects. Establishing a multidisciplinary diagnostic and therapeutic mode with neurosurgery as the core is of great significance for realizing the precise diagnosis and treatment of SFT and improving the prognosis of patients.