| Popis: |
Desmoid tumours (DT) are rare myofibroblastic monoclonal proliferations, which are present in 10-25 per cent of patients with familial adenomatous polyposis (FAP). FAP desmoids are frequently intra-abdominal desmoids (IAD) or abdominal wall desmoids (AWD) and rarely can be extra-abdominal desmoids (EAD). DT are non-metastatic, locally invasive and display a variable clinical course. The aims of the studies in this thesis were to further characterize FAP associated desmoids and identify predictors of aggressive phenotype using clinical, radiological and cell culture model. A meta-analysis of 4,625 FAP patients with 559 (twelve per cent) DT identified a positive family history of DT (odds ratio (OR) 7.02 95%CI 4.15-11.9, p |
