Desmoid

Desmoid

Aggressive fibromatosis is a rare condition marked by the presence of desmoid tumors, which are benign, slow-growing musculoaponeurotic tumors without any metastatic potential. However, aggressive fibromatosis is locally aggressive. Despite their benign nature, they can damage nearby structures causing organ dysfunction. Most cases are sporadic, but some are associated with familial adenomatous polyposis (FAP). Approximately 10% of individuals with Gardner's syndrome, a type of FAP with extracolonic features, have desmoid tumors.

Histologically they resemble low-grade fibrosarcomas, but they are very locally aggressive and tend to recur even after complete resection. There is a tendency for recurrence in the setting of prior surgery; in one study, two-thirds of patients with desmoid tumors had a history of prior abdominal surgery.

Risk factors for desmoid disease amongst FAP patients include female gender, a 3' APC mutation, a positive family history and a history of previous abdominal surgery.