Tenosynovial giant cell tumor is a rare proliferative tumor that arises from the synovium, bursae, or tendon sheaths due to an overproduction of colony-stimulating factor 1. Historically, treatment options for patients with local or diffuse tenosynovial giant cell tumor have been limited to surgical interventions. However, for some patients, surgical resection could worsen functional limitations and/or morbidity. In August 2019, the FDA approved pexidartinib (TURALIO™, Daiichi Sankyo), the first systemic treatment option for adult patients with symptomatic tenosynovial giant cell tumor associated with severe morbidity or functional limitations that were not amenable to improvement with surgery. Pexidartinib is an oral tyrosine kinase inhibitor with selective inhibition of colony-stimulating factor 1 receptor and is the first systemic therapy to show significant improvement in overall response rates when compared with placebo. Clinicians using pexidartinib should monitor for liver-related adverse events, which may require treatment interruption, dose reduction, or treatment discontinuation. Pexidartinib provides a novel non-surgical treatment option for patients with tenosynovial giant cell tumor that may significantly improve patients’ overall response, range of motion, physical function, tumor volume, and stiffness.

腱鞘巨细胞瘤是一种罕见的增生性肿瘤，由于集落刺激因子1的过量产生而由滑膜，法氏囊或腱鞘引起。历史上，局部或弥漫性腱鞘巨细胞瘤患者的治疗选择仅限于外科手术干预。但是，对于某些患者，手术切除可能会使功能限制和/或发病率恶化。在2019年8月，FDA批准了pexidartinib（TURALIO™，Daiichi Sankyo），这是针对患有严重腱鞘膜巨细胞瘤并伴有严重发病率或功能限制而无法通过手术改善的成年患者的第一种全身治疗选择。Pexidartinib是一种口服酪氨酸激酶抑制剂，具有选择性抑制集落刺激因子1受体的作用，是与安慰剂相比首个总体反应率显着改善的全身疗法。使用pexidartinib的临床医生应监测与肝脏相关的不良事件，这可能需要中断治疗，降低剂量或终止治疗。Pexidartinib为腱鞘巨细胞瘤患者提供了一种新型的非手术治疗选择，可显着改善患者的整体反应，运动范围，身体功能，肿瘤体积和僵硬程度。或停药。Pexidartinib为腱鞘巨细胞瘤患者提供了一种新型的非手术治疗选择，可显着改善患者的整体反应，运动范围，身体功能，肿瘤体积和僵硬程度。或停药。Pexidartinib为腱鞘巨细胞瘤患者提供了一种新型的非手术治疗选择，可显着改善患者的整体反应，运动范围，身体功能，肿瘤体积和僵硬程度。