Unlike traditional oncology where the goal is complete eradication, stable residual masses on imaging after successful desmoid tumor treatment are common and acceptable. Clinicians should not be compelled to operate or change therapy based on this finding alone, as it does not indicate active disease.
Clinicians should not underestimate the pain potential of desmoid tumors based on size. A case study showed a small 2.5cm tumor causing constant, debilitating pain that required nerve blocks for management, highlighting the poor correlation between tumor volume and symptom severity.
While Nirogacistat is a recent breakthrough, the next gamma-secretase inhibitor, Varegazistat, may be even more effective. Top-line data from its phase 3 trial showed a 56% response rate and an 84% reduction in progression risk, suggesting a rapidly advancing and competitive therapeutic landscape.
For desmoid tumors, systemic therapy is typically paused after 1 to 1.5 years once the tumor stabilizes and stops shrinking. Unlike many other treatments, it can be stopped abruptly without needing to taper the dose, simplifying the discontinuation process for patients and clinicians.
Surgical resection of desmoid tumors is now discouraged due to the risk of multifocal recurrence. A case demonstrated a patient developing multiple tumors throughout her leg 10 years after an initial surgery, illustrating how the tumor's finger-like growth can lead to widespread seeding if disturbed.