Desmoid-type fibromatosis: Current therapeutic strategies and future perspectives.

• Desmoid tumors are rare mesenchymal tumors with distinct clinical and molecular features. • Active surveillance is the initial approach for the management of desmoid tumors. • Hormone therapy, chemotherapy and targeted therapy are potential therapeutic approaches. • Active therapy might be indicated in symptomatic and or progressive DT. • Gamma secretase inhibitors constitute a promising therapy with survival benefit in DT. Desmoid tumors (DT) are rare, slow-growing, locally invasive soft tissue tumors that often pose significant therapeutic challenges. Traditional management strategies including active surveillance, surgery, radiotherapy, and systemic therapy which are associated with varying recurrence rates and high morbidity. Given the challenging nature of DT and the modest outcomes associated with current treatment strategies, there has been a growing interest in the field of γ-secretase inhibitors as a result of its action on the Wnt/β-catenin signaling pathway. In this review article, we will shed the light on the pathogenesis and molecular biology of DT, discuss its symptoms and diagnosis, and provide a comprehensive review of the traditional therapeutic approaches. We will also delve into the mechanisms of action of γ-secretase inhibitors, its efficacy, and the existing preclinical and clinical data available to date on the use of these agents, as well as the potential challenges and future prospects in the treatment landscape of these tumors. [ABSTRACT FROM AUTHOR]