Tenosynovial Giant Cell Tumour (TGCT)

Theme: Tenosynovial Giant Cell Tumour (TGCT)
Date:  12 March, 2021 (Fri), 19:00-20:40 HKT

Topic 1: PVNS/TGCT – When to treat and how to treat?
Presenter: Dr. Chao-Ming Chen, Attending Orthopaedic Surgeon, Department of Orthopaedics Surgery & Traumatology, Taipei Veterans General Hospital, Taiwan

Abstract:
Tenosynovial giant cell tumor (TGCT or TSGCT) is a family of lesions usually involving the joint synovia, bursae and tendon sheath. It may be intra- or extra-articular, and is classified as localized or diffuse lesions. While any locations might be involved, the localized TGCT mainly involve the fingers (up to 85% of all localized cases) and knee joints, followed by hip and ankle joints. The diffuse TGCTs mainly involve the large joints, such as knee, ankle, hip and elbow. The localized forms are systematically benign whereas the diffuse types are more aggressive and destructive and may exceptionally include a malignant component. Sometimes, it is also known as pigmented villonodular synovitis (PVNS), especially when the lesions involve knee and hip joints. Because of the great diversity of clinical presentations and biological behaviors, many therapeutic strategies have been proposed, and there is no single treatment superior to the other. Here, we would like to review pathogenic mechanism of the disease and introduce different treatment strategies aimed to relieve symptoms, control disease progression, and preserve more function for the patients.

Topic 2: Practical aspects of systemic therapies in the management of PVNS/TGCT
Presenter: Dr. Tom Wei-Wu Chen, Attending Physician, Department of Oncology, National Taiwan University Cancer Center, Taiwan

Abstract:
Tenosynovial giant cell tumor (TGCT), or sometimes called pigmented villousnodular synovitis (PVNS), is a neoplastic mesenchymal tumor occur in the tendon sheath. The incidence is very low, but diffuse type TGCT may cause severe morbidity if surgery is not amenable. TCGT neoplastic cells are characterized by the presence ofCOL6A3-CSF1A gene rearrangement. The excessive colony-stimulating factor 1 (CSF1) attracts macrophages to the tumor and fuels the growth of the tumor. Recent studies have shown that CSF-1R inhibitor pexidartinib is a viable option for the treatment of TGCT. We will discuss in detail the medical treatment in detail for TGCT in this lecture.