Clinical Presentation

Desmoids can remain asymptomatic for a considerable length of time, all the while relentlessly enlarging and infiltrating adjacent structures. However, DTs may show a capricious, variable clinical behaviour, usually characterized by an indolent course, rarely by a spontaneous regression and sometimes by an aggressive and rapid growth and a tendency to invade surrounding structures. Desmoid tumours may cause abdominal pain, nausea, vomiting, diarrhoea and deterioration of the functional result in patients submitted to restorative surgery after total colectomy. The intra-abdominal tumour growth may induce small-bowel obstruction or other life-threatening complications such as intestinal perforation or intestinal infarct as the result of the compression of the blood vessels which may impair vascular supply and cause small-bowel ischaemia or mesenteric thrombosis [14, 16]. The consequences of mucosal ischaemia of the small bowel are bleeding or intestinal strictures [21]. Sudden enlargement of the DT can provoke deep vein thrombosis and fatal pulmonary embolism [22]. The tumoral mass may undergo colliquative necrosis and abscess formation which can determine an abdominal emergency or a spontaneous discharge into the intestinal lumen with fistula formation. Also mono- or bilateral hydronephrosis as a result of retroperitoneal invasion can be observed.

Desmoid tumours are the second most common cause of death in FAP patients after colorectal cancer. Intra-abdominal DTs can be responsible for death in up to 11% of FAP patients [23-25]. In the experience of the Johns Hopkins University, the survival rate from DTs evaluated by life-table analysis is 93% at 5 years and 79% at 20 years with a mean age of death in the affected patients of 40 years [2].

Patients who have had an ileal pouch-anal anastomosis (IPAA) and have developed a DT, show a worse functional result than IPAA patients not developing a DT [26, 27]. The occurrence of small-bowel obstruction or intestinal bleeding in IPAA patients with DTs usually requires the removal of the pouch [21,26].

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