Abstract
Desmoid disease results when the wnt signaling pathway in fibroblasts is constantly active. It is characterized by enlarging tumors and sclerosing sheets of tissue. In patients with familial adenomatous polyposis (FAP) this can happen in the abdomen, the abdominal wall and in extra-abdominal sites. This review describes the clinical sequelae of desmoid disease in patients with FAP and their management. While abdominal wall desmoids cause symptoms due to their increasing size and prominence, they can be resected with a recurrence rate of about 30%. Intra-abdominal desmoid disease often involves the small bowel mesentery and cannot be resected without enterectomy. Various medical options are available for their management but none are predictably successful. A staging system has been developed that predicts mortality and allows rational treatment choices. Specific complications such as obstruction of the ureter and small bowel, small bowel perforation with abscess and fistula, and hemorrhage may need surgical management. Most FAP patients with desmoid disease can live in equilibrium with the desmoids. The keys are to mitigate symptoms while avoiding toxic or dangerous therapy.
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