Solitary fibrous tumors (SFT) are rare mesenchymal neoplasms that account for less than 2% of all soft tissue masses. In the latest WHO 2020 Classification of Soft Tissue Tumors, extrameningeal SFT was listed as intermediate (rarely metastasizing) or malignant neoplasms. Due to the lack of characteristic clinical features, their diagnosis and treatment remain challenging. The pathogenesis of SFT is often associated with the presence of fusions of the NAB2-STAT6 gene on the 12q13 chromosome. Cytoplasmic CD34 positive staining is considerably characteristic for most SFTs; less frequently, factor XII, vimentin, bcl-2, and CD99 are present. A key factor in the diagnosis is the prevalent nuclear location of STAT6 expression. Radical resection is the mainstay of localized SFTs. In the case of unresectable disease, only radiotherapy or radio-chemotherapy may significantly ensure long-term local control of primary and metastatic lesions. To date, no practical guidelines have been published for the treatment of advanced or metastatic disease. Classical anthracycline-based chemotherapy is applicable. The latest studies suggest that antiangiogenic therapies should be considered after first-line treatment. Other drugs, such as imatinib, figitumumab, axitinib, and eribulin, are also being tested. Definitive radiotherapy appears to be a promising therapeutic modality. Since standards for the treatment of advanced and metastatic diseases are not available, further investigation of novel agents is necessary.
孤立性纤维瘤是一种罕见的间叶组织肿瘤，占所有软组织肿物的比例不足2%。在最新的WHO 2020软组织肿瘤分类中，脑膜外孤立性纤维瘤被列为中间型（罕见转移）或恶性肿瘤。由于缺乏特征性临床表现，其诊断与治疗仍具挑战性。该病的发病机制常与12q13染色体上NAB2-STAT6基因融合相关。细胞质CD34阳性染色对大多数孤立性纤维瘤具有显著特征性；较少出现XII因子、波形蛋白、bcl-2及CD99表达。诊断的关键因素在于STAT6表达主要定位于细胞核。根治性切除术是局限性孤立性纤维瘤的主要治疗手段。对于不可切除病例，仅放疗或放化疗可能显著确保原发灶和转移灶的长期局部控制。迄今为止，尚未发布针对晚期或转移性疾病的实用治疗指南。传统蒽环类化疗方案具有适用性。最新研究表明一线治疗后应考虑抗血管生成治疗。其他药物如伊马替尼、非格司亭单抗、阿昔替尼和艾日布林也正在临床试验中。根治性放疗显示出成为有前景治疗模式的潜力。鉴于晚期及转移性疾病缺乏标准化治疗方案，有必要进一步研究新型治疗药物。
Diagnostics and Treatment of Extrameningeal Solitary Fibrous Tumors
肿瘤(癌症)患者之家