Research Progress on Desmoid Tumors: New Perspectives from Molecular Mechanisms to Clinical Management

Summary

Desmoid Tumors (DT) are rare fibromatoses that are locally invasive. These are described as being monoclonal, and they tend to recur. Their clinical features are also different, and the prognoses of these tumors are not predictable. The recent achievements made in molecular genetics and the encouragement of multidisciplinary treatment modes have made a tremendous contribution to the diagnosis, treatment strategies, and qualityof-life measures of patients with DT. This is a narrative review that summarizes clinical, molecular pathology, conventional, and new areas of treatment, prognosis, and the current research on quality of life assessment tools in DT. It specifically highlights the uses and opportunities of targeted therapies, such as the secretase inhibitor nirogacestat (DeFi trial, NCT03785964) and tyrosine kinase inhibitors, such as sorafenib, reporting substantial disease progression, and critically reviews the role of surgery, in the context of high recurrence rates of marginpositive or even margin-negative resection. The study indicates the significance of personalized treatment that varies treatment options with proper risk-benefit analysis of each patient. This review also explains that the heterogeneous nature of DT and the absence of a standard-of-care predetermine the necessity of such an individual approach. Future areas of research that would offer a more powerful scientific foundation for individual guidance are also mentioned.