Abstracts
1998 Digestive Disease Week

#997

DESMOID TUMOURS IN FAMILIAL ADENOMATOUS POLYPOSIS (FAP). C. Soravia, T. Berk, R.S. McLeod, Z. Cohen, Department of Surgery, Familial Gastrointestinal Cancer Registry, Mount Sinai Hospital, University of Toronto, Toronto, Canada.

The purpose of this retrospective study was to review the clinical presentation, surgical management and outcome of FAP patients with desmoid tumours (DTs). DTs were documented in 97 out of 780 FAP patients (12.4%). Demographic (including family history), operative and follow-up data were retrieved from the FAP registry. Over-all, there were 38 males (mean age 32.1 years) and 59 females (mean age 29.1 years). A family history of DTs was found in 41 patients (42.2%). The most common clinical manifestation was small bowel obstruction, occurring in 49% of those with intra-abdominal DTs alone (48) and intra-abdominal and abdominal wall DTs (28). DT diagnosis was made after colorectal surgery in 77 cases (79.3%). Average time between colon and DT surgery was 4.6 years (range 8 months to 38 years). 21 patients have not required surgery for their DTs. 8 patients died of their intra-abdominal DTs. Ileo-rectal anastomosis was the most common initial prophylactic procedure (59 patients). 11 had a pelvic pouch and 15 a total proctocolectomy. Conversion from an IRA was necessitated in 26 patients because of uncontrollable rectal polyps (23) or cancer (3). 9 of these 26 could not be converted to a pelvic pouch or a Kock pouch for technical reasons. Only 1 patient had total removal of a DT and subsequently died following small bowel transplantation. 42 patients were treated with Sulindac and/or Tamoxifen, with symptomatic improvement in only 2 cases. Cytotoxic chemotherapy was reserved for the most complex cases, but 6 of those were improved significantly with 2 being stable and 2 worse. In summary, DTs were found in 10% of our FAP patients. DT behaviour was not influenced by prior family history. The most common indication for surgery was small bowel and/or ureteric obstruction without total removal of the DT. Conversion following an initial IRA may be impossible due to technical reasons, and therefore consideration should be given to doing a pelvic pouch as the initial procedure in these patients. Non-cytotoxic medical therapy has not been effective in our patient population, but cytotoxic chemotherapy has been very effective in selected complex cases.

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