孤立性纤维性肿瘤(SFT)是一种罕见的成纤维细胞间充质肿瘤。目前的分类已将SFT和血管外皮细胞瘤(HPC)合并为同一肿瘤实体,而风险分层模型已经开发出来,以补偿临床预测。通常,生长缓慢且无症状,SFT可以发生在各种解剖部位,最常见于胸膜.组织学上,SFT由纺锤体到卵圆形细胞组成,具有最小的模式生长,被基质胶原蛋白和独特的血管模式包围。分子上,SFT由NGFI-A结合蛋白2(NAB2)和信号转导和转录激活因子6(STAT6)基因的融合定义为NAB2-STAT6。这种融合将NAB2转化为转录激活因子,激活早期生长反应1(EGR1)并促进SFT的发病和发展。在肿瘤组织中有几种NAB2-STAT6的融合变体,最常见的是NAB2ex4-STAT6ex2和NAB2ex6-STAT6ex16/ex17。诊断方法在SFT临床实践和基础研究中起着至关重要的作用,包括RT-PCR,下一代测序(NGS),FISH,免疫组织化学(IHC),和蛋白质印迹分析,每个都有不同的能力和局限性。SFT的传统治疗策略包括手术切除,放射治疗,和化疗,虽然新兴的管理制度包括抗血管生成药物,免疫疗法,RNA靶向技术,和潜在的靶向药物。这篇综述提供了SFT的临床和分子方面的最新信息,诊断方法,和潜在的疗法。
Solitary fibrous tumor (SFT) is a rare fibroblastic mesenchymal neoplasm. The current classification has merged SFT and hemangiopericytoma (HPC) into the same tumor entity, while the risk stratification models have been developed to compensate for clinical prediction. Typically, slow-growing and asymptomatic, SFT can occur in various anatomical sites, most commonly in the pleura. Histologically, SFT consists of spindle to oval cells with minimal patterned growth, surrounded by stromal collagen and unique vascular patterns. Molecularly, SFT is defined by the fusion of NGFI-A-binding protein 2 (NAB2) and signal transducer and activator of transcription 6 (STAT6) genes as NAB2-STAT6. This fusion transforms NAB2 into a transcriptional activator, activating early growth response 1 (EGR1) and contributing to SFT pathogenesis and development. There are several fusion variants of NAB2-STAT6 in tumor tissues, with the most frequent ones being NAB2ex4-STAT6ex2 and NAB2ex6-STAT6ex16/ex17. Diagnostic methods play a crucial role in SFT clinical practice and basic research, including RT-PCR, next-generation sequencing (NGS), FISH, immunohistochemistry (IHC), and Western blot analysis, each with distinct capabilities and limitations. Traditional treatment strategies of SFT encompass surgical resection, radiation therapy, and chemotherapy, while emerging management regimes include antiangiogenic agents, immunotherapy, RNA-targeting technologies, and potential targeted drugs. This review provides an update on SFT\'s clinical and molecular aspects, diagnostic methods, and potential therapies.