Abstract

Desmoid tumours are dense fibroblastic tumours occurring in any mesenchymal tissue at the site of a fascia. It is usual to distinguish extra-abdominal and abdominal forms, although they are microscopically similar. The extra-abdominal variety is also called aggressive fibromatosis (AF). Abdominal desmoid involves either the abdominal wall, particularly the lower part of the rectus abdominis muscle, or the abdominal cavity where it arises in the mesentery or from any muscular structure. The abdominal variety tends to occur more frequently in women of child-bearing age. This review will only focus on the extra-abdominal variety. AF is a tumour with many contrasts: it is a purely benign process but it may infiltrate vital structures and cause severe morbidity. The growth potential can be high in some cases, with early recurrence, whereas spontaneous regression can be observed in others. Owing to such a disconcerting tumoral behaviour, the treatment has been a matter for debate and is the subject of this article.