The term desmoid was first used by Muller [1] to describe the tendon-like aspect and the hard consistency of this type of proliferation (desmos in Greek means band). Desmoid tumours (DTs) are classified as extraor intra-abdominal. The extra-abdominal DTs arise from fascial or musculoaponeurotic structures predominantly of the abdominal wall (Fig. 1a, b, c) and occasionally of the shoulder girdle, chest wall, inguinal region and extremities. They present as a firm, smooth, painless, progressively growing mass. Imaging investigations are useful in better defining the extent of the tumour which displays an iceberg growth with only a small proportion being clinically manifest. Interestingly, multiple extra-abdominal DTs are frequently discovered in very young patients under the age of 3 [2]. The intra-abdominal DTs develop in the folds of the mesentery or the mesocolon (Fig. 2a, b) even reaching the retroperitoneal tissue or they may grow exclusively in this region. These proliferations are usually single, round or oval in shape and up to 60 cm in size. Rather than a mass, a thickening of the mesentery that appears to be covered with hard white spots and causes retraction of the peritoneal folds is frequently reported in familial Desmoid Tumours in Familial Adenomatous Polyposis
