Department of Orthopaedic Surgery, David Geffen School of Medicine, UCLA, Los Angeles, CA, 90055, USA.
Curr Oncol Rep. 2020 Jun 4;22(6):63. doi: 10.1007/s11912-020-00926-7.
Pigmented villonodular synovitis (PVNS) or tenosynovial giant cell tumor (TGCT) encompasses a wide spectrum of disease and is divided into localized and diffuse variants. Surgical resection remains the principal treatment for nearly all localized type disease and most diffuse type. Recent mechanistic understanding of the disease led to drug discovery that has opened new avenues for patients with recalcitrant disease. In this manuscript, we review the current treatment options for TGCT, presenting outcomes from traditional surgical approaches as well as those from nonsurgical approaches.
Arthroscopic and/or open surgery remains the mainstay of treatment for TGCT for the vast majority of patients. While radiosynoviorthesis and external beam radiation have been used for recalcitrant disease, recent understanding of the colony stimulating factor 1 receptor (CSF1R) pathway and its paracrine and autocrine role in TGCT has led to the development of targeted inhibitors. Their optimal role and efficacy are unclear due to limited number of high-quality studies and contradictory results; however, recent and ongoing studies suggest there may be a role for their use, especially in diffuse and/or refractory disease. Surgery remains the most common treatment for TGCT, however, there may be an increasing role for adjuvant therapies, including the new targeted agents. Weighing the side effects of these treatments against the symptomatic benefit on a patient-by-patient basis in this benign disease remains critical.
色素绒毛结节性滑膜炎（PVNS）或腱鞘巨细胞瘤（TGCT）涵盖了广泛的疾病谱，分为局限性和弥漫性两种类型。手术切除仍然是治疗几乎所有局限性疾病和大多数弥漫性疾病的主要方法。对疾病机制的最新认识导致了药物的发现，为顽固疾病患者开辟了新的治疗途径。在本文中，我们回顾了 TGCT 的当前治疗选择，介绍了传统手术方法和非手术方法的结果。
关节镜和/或开放性手术仍然是绝大多数 TGCT 患者的主要治疗方法。虽然放射性滑膜切除术和外照射放疗已用于治疗顽固疾病，但最近对集落刺激因子 1 受体（CSF1R）途径及其在 TGCT 中的旁分泌和自分泌作用的认识导致了靶向抑制剂的发展。由于高质量研究数量有限且结果相互矛盾，其最佳作用和疗效尚不清楚；然而，最近和正在进行的研究表明，它们的使用可能有一定的作用，特别是在弥漫性和/或难治性疾病中。手术仍然是 TGCT 最常见的治疗方法，但辅助治疗，包括新的靶向药物，可能会发挥越来越大的作用。在这种良性疾病中，根据每位患者的症状获益和副作用来权衡这些治疗方法仍然至关重要。
