Answer

From question on page 15

The biopsy was consistent with desmoid tumour. Desmoid tumours are one of the extracolonic manifestations of familial adenomatous polyposis (FAP). They represent the most common cause of death in FAP after colorectal cancer. The incidence of desmoid tumours in patients with FAP varies between 10% and 18%. Three major factors seem to play a role in the development of desmoid tumours: surgical trauma, oestrogen, and genetic factors. Despite their benign histopathological appearance, these tumours can be very aggressive and locally invasive. The clinical presentation of desmoid tumours varies from an asymptomatic abdominal wall mass, to bowel and ureteral obstruction, perforation, or haemorrhage. Medical treatment with anti-oestrogens, steroids, colchicine, interferon α, and warfarin has been tried with minimal success. Chemotherapy has been used with promising results. Surgery should be limited to acute or chronic small bowel obstruction because of the high rate of recurrence.