孤立性纤维性肿瘤
医学
血管外皮细胞瘤
肉瘤
放射治疗
软组织肉瘤
软组织
鉴别诊断
疾病
局限性疾病
病理
肿瘤科
放射科
内科学
癌症
生物
川地34
遗传学
前列腺癌
干细胞
作者
Alannah Smrke,Khin Thway,Paul H. Huang,Robin L. Jones,Andrew Hayes
出处
期刊:Future Oncology
[Future Medicine]
日期:2021-09-01
卷期号:17 (27): 3627-3636
被引量:10
标识
DOI:10.2217/fon-2021-0030
摘要
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 NAB2-STAT6 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.
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