Sarcoma Unit, Royal Marsden Hospital, 203 Fulham Road, London, SW3 6JJ, UK.
The Institute of Cancer Research, 237 Fulham Road, London, SW3 6JB, UK.
Future Oncol. 2021 Sep;17(27):3627-3636. doi: 10.2217/fon-2021-0030. Epub 2021 Aug 19.
Solitary fibrous tumor (SFT) is a rare soft tissue sarcoma subtype which mainly affects adults in the fifth and sixth decades of life. Originally part of a spectrum of tumors called hemangiopericytomas, classification has been refined such that SFTs now represent a distinct subtype. The identification of fusion in virtually all SFTs has further aided to define this rare subgroup. SFTs have a spectrum of behavior from benign to malignant, with evidence suggesting risk of metastases related to age at diagnosis, extent of necrosis, mitotic rate and tumor size. The standard treatment for localized disease is surgical excision with or without radiotherapy. Retrospective and prospective evidence suggests antiangiogenic treatment is effective for unresectable disease. Further translational work is required to understand the biology driving the differential behavior and identify more effective treatments for patients with metastatic disease.
孤立性纤维瘤（SFT）是一种罕见的软组织肉瘤亚型，主要影响 50-60 岁的成年人。它最初是血管外皮细胞瘤的一种，随着分类的细化，SFT 现在代表了一种独特的亚型。几乎所有 SFT 中融合的鉴定进一步帮助定义了这个罕见的亚组。SFT 的行为从良性到恶性不等，有证据表明转移风险与诊断时的年龄、坏死程度、有丝分裂率和肿瘤大小有关。局限性疾病的标准治疗是手术切除加或不加放疗。回顾性和前瞻性证据表明，抗血管生成治疗对不可切除的疾病有效。还需要进一步的转化研究来了解驱动不同行为的生物学特性，并为转移性疾病患者找到更有效的治疗方法。
