Unresectable desmoid tumours causing obstructive uropathy in familial adenomatous polyposis

Desmoid tumour-related ureteral obstruction in familial adenomatous polyposis presents difficult management problems. Its role in altering prognosis for the worse in young patients has been neglected. Two patients are reported and 14 cases in the literature reviewed. Both our patients developed intra-abdominal desmoid tumours following proctocolectomies with pouch reconstruction (performed 0.7 and 2.5 years earlier). Despite therapy with sulindac and tamoxifen, both suffered progressive ureteral obstruction that required initial percutaneous drainage. Ureteral stenting was effective and safe in both patients (10 and 18 months' follow-up, respectively), but the long-term outcome awaits assessment. The optimal regimen of adjuvant medical therapy is uncertain. Successful renal autotransplantation and combination chemotherapy have been tried after failure of conventional therapy.