Desmoid tumour: a pleomorphic lesion

Background: Desmoid tumour (DT) is an uncommon locally invasive non-metasta sizing neoplastic lesion. The aetiology of this tumour is unknown and its t reatment is controversial. Twelve cases of DT are presented and the literat ure is reviewed. Methods: Twelve cases of DT treated at our institution during a 3.5-year pe riod are analysed and the literature reviewed. Ten patients were referred w ith a primary tumour, one with local recurrence and one patient with a seco nd primary desmoid tumour. One patient had multiple mesenteric DT (familial adenomatous polyposis coli-FAP), and in the remaining 11 patients the tumo ur was located in the abdominal wall in four, at an extremity in three, in the upper back in two patients, in the pelvis in one and retroperitoneally in one. Results: The largest mesenteric DT was marginally excised en bloc with tota l jejunectomy. In the remaining 11 DT. complete excision to microscopically tumour-free margins was possible in nine cases and to microscopically invo lved margins in two cases. At a mean follow-up of 22 months (range 7-38 mon ths), one patient was alive with stable disease (Gardner's syndrome), 10 pa tients were alive and free of recurrence and one patient (9%) developed loc al recurrence which was re-excised-she is disease-free 10 months later. Conclusions: Complete excision is the main modality of treatment for primar y and recurrent DT. This is feasible in most cases except for tumours invol ving the base of the bowel mesentery. Surgical resection alone achieved loc al control of the tumour in most of the patients in this series (92%).