Current Treatment Strategies for Diffuse Tenosynovial Giant Cell Tumor: A Review of the Literature.

Background: Diffuse tenosynovial giant cell tumor (DTGCT) is a locally aggressive benign tumor of the synovium. Patients often initially present with pain, stiffness, and swelling of the affected joint with varying levels of severity. Treatment traditionally involved surgical resection exclusively; however, this could be complicated by high disease recurrence rates. New research has introduced several targeted systemic therapies onto the market changing the treatment paradigm and necessitating a multidisciplinary treatment approach in specialized centers to optimize patient outcomes. Methods: This review synthesizes the current literature on DTGCT including its pathophysiology, classification, diagnosis, and available treatment options. There is a particular focus on the newer systemic therapies available and how these medications may be used in conjunction with surgery to enhance disease control. Results: DTGCT most commonly affects young to middle-aged adults, with a slight female predominance, and is most frequently found in the knee. Arthroscopic and even open synovectomy can have disease recurrence rates exceeding 50%. Colony stimulating factor 1 (CSF1) receptor inhibitors have proven effective at symptom palliation and reducing tumor burden in approximately 40% of patients. While these medications improve the quality of life for patients with unresectable disease, they may also be effective in the neoadjuvant setting to downstage surgical approaches and possibly improve disease control in otherwise highly morbid cases. Conclusions: Surgery alone, the traditional standard for DTGCT, is often insufficient due to high recurrence rates. Systemic therapies can restore function and improve quality of life in patients with advanced disease with rare-but potentially serious-adverse effects. Combining surgical resection with neoadjuvant CSF1R inhibition may provide superior outcomes. Further research is needed to refine the role of systemic agents and develop multidisciplinary protocols. Although initial symptoms often lead patients to community providers, optimal care for patients with DTGCT is best delivered at referral centers with dedicated musculoskeletal oncology programs. Level of Evidence: Level V. See Instructions for Authors for a complete description of levels of evidence.