Tenosynovial giant cell tumour: from active surveillance to surgery and systemic therapy

Abstract

This thesis investigates the diffuse form of Tenosynovial Giant Cell Tumor (D-TGCT), a rare tumor predominantly affecting relatively young individuals. It highlights the complexity of D-TGCT, detailing the patient journey from diagnosis to evaluating treatment options. The Tenosynovial Giant Cell Tumour Observational Platform Project (TOPP) is the first prospective disease registry for TGCT. It reveals that diagnosis often takes years and that D-TGCT significantly impacts patients' daily lives. MRI scans are essential for diagnosing D-TGCT, and a structured assessment protocol has been developed.

Surgery is generally the primary treatment, but a watchful waiting approach may be suitable for some. Surgical resection carries a high recurrence risk, and incomplete removal can lead to disease progression. Complete resection is advised but may not always be feasible without causing significant harm to the body.

Systemic therapies, such as CSF1R-targeted treatments, offer new possibilities for patients unable or unwilling to undergo surgery. While these therapies show promise, further research is needed to understand their long-term effects. The benefits of systemic treatments must be weighed against potential side effects. This thesis emphasizes the importance of multidisciplinary collaboration for managing this complex disease.