Tailored surgical treatment of duodenal polyposis in familial adenomatous polyposis syndrome.

BACKGROUND

To review our experience in patients undergoing operative treatment for duodenal polypoisis associated with familial adenomatous polyposis with an emphasis on operative approach and long-term outcomes.

METHODS

Duodenal polypoisis associated with familial adenomatous polyposis patients undergoing operative treatment were studied retrospectively excluding patients with preoperative duodenal cancer.

RESULTS

Of 767 patients in the database, 63 (8.2%) patients underwent operative treatment: 42 (67%) pancreas-sparing duodenectomy, 15 (24%) pancreatoduodenectomy, and 6 (9.5%) segmental duodenal resection; the majority for Spigelman stages III and IV polyposis. Overall 9.6% had adenocarcinoma postoperatively (28.6% in the pancreatoduodenectomy group; P = .01). The proportion of Spigelman stages III and IV with cancer were 9.5% and 6.5%, respectively. Pathologic upgrade to cancer in patients with low grade dysplasia and high-grade dysplasia on preoperative biopsy was 5.7% and 6.7%, respectively (P = .13). At a median follow-up of 16 years, 7.7% needed a second duodenal polypoisis associated with familial adenomatous polyposis-related operation. Progression to high grade dysplasia or cancer in the stomach occurred in 15.4% of patients. Median overall survival and recurrence-free survival was at least 16 years and 15.6 years. No significant group-based differences were noted on follow-up.

CONCLUSION

The majority of patients with duodenal polypoisis associated with familial adenomatous polyposis can achieve long-term, cancer-free survival with organ-preserving approaches (pancreas-sparing-duodenectomy and segmental-duodenal-resection) with survival not dependent on the type of resection.