目的：回顾性分析颞下颌关节弥漫型腱鞘巨细胞瘤（diffuse tenosynovial giant cell tumor，D-TSGCT）的临床特点及治疗预后，为该病的早期诊断和治疗提供参考。方法： 收集2003年10月至2015年8月期间在北京大学口腔医院就诊并进行手术治疗，最终组织病理诊断为颞下颌关节D-TSGCT的患者15例，就其临床表现、影像学及组织病理学特点、诊断与鉴别诊断、治疗和随访情况等进行总结和讨论。结果： 15例中12例为女性，主要症状包括耳前区疼痛性肿胀或肿块、开口受限及开口偏斜等。CT表现主要为关节区软组织肿块，增强时有强化，常伴有髁突、关节窝及颅底骨质破坏，关节间隙增宽。MRI检查11例，6例在T1、T2加权序列上均呈现较低信号表现。9例病变向关节外扩展，6例有颅底破坏，病变进入颞下窝、颅中窝者各有4例。入院后行手术切除者14例，手术活组织检查者1例，术后接受放射治疗者3例，术后随访有3例复发。结论： 颞下颌关节D-TSGCT应注意与颞下颌关节紊乱病、颞下颌关节其他肿瘤及瘤样病变以及腮腺区肿瘤等进行鉴别。CT及MRI检查对D-TSGCT的诊断和治疗设计有重要价值。因该病有侵袭性，可扩展到关节外，特别是对有颅底破坏者，应尽快彻底手术切除。术后放射治疗对于病变范围广、有颅底破坏者是一种较好的补充治疗。D-TSGCT存在复发和恶变的可能性，建议长期随访。

Objective：To retrospectively analyze the clinical features, treatment and prognosis to the diffuse tenosynovial giant cell tumor (D-TSGCT) arising from the temporomandibular joint (TMJ), and to give a reference for the early diagnosis and treatment of this disease. Methods: In this study, 15 patients finally diagnosed as D-TSGCT of TMJ histopathologically at the Peking University Hospital of Stomatology from October 2003 to August 2015 were selected and reviewed. Their clinical manifestations, imaging and histological features, diagnoses and differential diagnoses, treatments and follow-ups were summarized and discussed. Results: D-TSGCT of TMJ showed obvious female predominance (12/15), the main symptoms included painful preauricular swelling or mass, limited mouthopening and mandibular deviation with movement. D-TSGCT on computed tomography (CT) scan often showed illdefined soft tissue masses around TMJ, enhancement after contrast administration, usually with widening of the joint spaces and with bone destruction of the condyle, the fossa and even the skull base. On magnetic resonance images (MRI), the majority of lesions on T1 weighted images and T2 weighted images both showed the characteristics of low signals (6/11). The lesions could extend beyond the joints (9/11) and into the infratemporal fossa (4/11) and the middle cranial fossa (4/11). Surgical resection was performed in 14 cases and biopsy in 1 case. Postoperative radiotherapy was performed in 3 cases. In follow-ups, 3 cases showed recurrence postoperatively. Conclusion: D-TSGCT arising from TMJ should be differentiated with TMJ disorders, other tumors and tumor-like lesions of TMJ and parotid neoplasms, etc. CT and MRI examinations have important values in the diagnosis and treatment design of DTSGCT. Because of the local aggressive and extensive behavior, complete resection should be performed as soon as possible. Postoperative radiotherapy was helpful for the extensive lesions including destruction of skull base and may be a good supplementary therapy. Because of the possibility of recurrence and malignancy, long-term follow-up was suggested.