摘要
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.关键词
孤立性纤维瘤/中枢神经系统肿瘤/诊断/综合治疗/预后Key words
solitary fibrous tumor/central nervous system neoplasms/diagnosis/combined-modality therapy/prognosis分类
医药卫生
