Surgical management for advanced duodenal adenomatosis and duodenal cancer in Dutch patients with familial adenomatous polyposis: A nationwide retrospective cohort study

BWH van Heumen, MH Nieuwenhuis, H van Goor, EMH Mathus-Vliegen, E (Erwin) Dekker, DJ Gouma, Jan Dees, Casper van Eijck, HFA Vasen, FM Nagengast

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Background. Duodenal cancer is a major cause of mortality in patients with familial adenomatous polyposis (FAP). The clinical challenge is to perform duodenectomy before cancer develops; however, procedures are associated with complications. Our aim was to gain insight into the pros and cons of prophylactic duodenectomy. Methods. Patients with FAP from the nationwide Dutch polyposis registry who underwent prophylactic duodenectomy or were diagnosed with duodenal cancer were identified and classified as having benign disease or cancer at preoperative endoscopy. Surveillance, clinical presentation, surgical management, outcome, survival, and recurrence were compared. Results. Of 1,066 patients with FAP in the registry, 52 (5%; 25 males) were included: 36 with benign adenomatosis (median: 48 years old; including two (6%) cancer cases diagnosed after operation), and 16 with cancer (median: 53 years old). Cancer cases had been diagnosed with colorectal cancer more often (6% vs 44%; P < .01). Forty-three patients underwent duodenectomy (35 benign/eight cancer): 30-day mortality was 4.7% (n = 2), and in-hospital morbidity occurred in 21 patients (49%), without di Conclusion. Prognosis of duodenal cancer in. patients with FAP is poor, which justifies an aggressive approach to advanced benign adenomatosis. Strict adherence to recommended surveillance intervals is essential for a well-timed intervention. Given the substantial morbidity and mortality of duodenectomy, patients' individual characteristics are to be critically evaluated preoperatively. As adenomas recur, postoperative endoscopic surveillance is mandatory. (Surgery 2012;151:681-90.)
Original languageUndefined/Unknown
Pages (from-to)681-690
Number of pages10
Issue number5
Publication statusPublished - 2012

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