Palmerini Emanuela, Trent Jonathan C, Hornicek Francis John
Sylvester Comprehensive Cancer Center, University of Miami, 1475 NW 12th Ave, Miami, FL, 33136, USA.
Miller School of Medicine, University of Miami, Miami, FL, USA.
Curr Oncol Rep. 2025 May 20. doi: 10.1007/s11912-025-01679-x.
Diffuse tenosynovial giant cell tumor (D-TGCT) is a benign neoplasm with locally aggressive potential of the synovium, bursae, and tendon sheaths. This review summarizes the current treatment landscape for D-TGCT, with a focus on systemic therapies.
Surgery is the primary treatment option for tenosynovial giant cell tumor (TGCT), but there is a high risk of recurrence and associated morbidity, particularly for patients with advanced D-TGCT. Systemic therapies targeting the colony-stimulating factor 1 receptor (CSF1R) have resulted in positive tumor response, improved function, and decreased symptoms. For an alternative to surgery, the CSF1R inhibitors pexidartinib and vimseltinib are approved in the United States for TGCT, and other CSF1R inhibitors are in clinical development. CSF1R inhibitors represent a significant evolution in therapeutic strategies for D-TGCT. The potential risks and benefits of available treatments should be carefully considered in collaboration with a bone tumor-experienced, multidisciplinary team to determine the best course of care. Increased D-TGCT awareness and support through patient advocacy groups have helped to reshape the patient journey.
弥漫性腱鞘巨细胞瘤（D-TGCT）是一种起源于滑膜、滑囊和腱鞘的具有局部侵袭性的良性肿瘤。本综述总结了D-TGCT的当前治疗情况，重点关注全身治疗。
手术是腱鞘巨细胞瘤（TGCT）的主要治疗选择，但复发风险和相关并发症发生率较高，尤其是晚期D-TGCT患者。靶向集落刺激因子1受体（CSF1R）的全身治疗已产生了积极的肿瘤反应、改善了功能并减轻了症状。作为手术的替代方案，CSF1R抑制剂培西达替尼和维姆塞替尼在美国已被批准用于TGCT治疗，其他CSF1R抑制剂也正在进行临床开发。CSF1R抑制剂代表了D-TGCT治疗策略的重大进展。应与经验丰富的骨肿瘤多学科团队合作，仔细权衡现有治疗方法的潜在风险和益处，以确定最佳治疗方案。通过患者倡导组织提高对D-TGCT的认识并提供支持，有助于重塑患者的就医过程。
