Desmoid disease in familial adenomatous polyposis

Desmoid tumors are clonal, locally aggressive fibroblastic neoplasms that lack metastatic potential but are a major source of morbidity and mortality in patients with familial adenomatous polyposis (FAP). They occur in approximately 10–25% of individuals with FAP, typically developing within a few years after colectomy, and arise mainly from the small bowel mesentery. Integration of genotype, family history, and prior surgical exposure into structured risk assessment tools can inform the timing and extent of colorectal surgery, surveillance strategies, and earlier consideration of systemic therapy. Management is largely dictated by clinical behavior. Active surveillance is the preferred first-line strategy, except in tumors located in critical sites, associated with complications, or demonstrating aggressive progression during follow-up, in which case active treatment including systemic therapy and/or surgical intervention should be considered.