TITLE

WHEN IS PROPHYLACTIC SURGERY FOR DUODENAL ADENOMATOSIS IN FAP JUSTIFYABLE?

AUTHOR(S)
Gallagher, M.C.; Phillips, R.K.S.
PUB. DATE
April 2003
SOURCE
Gut;Apr2003 Supplement 1, Vol. 52, pA16
SOURCE TYPE
Academic Journal
DOC. TYPE
Article
ABSTRACT
Background: Despite > 90% of patients with familial adenomatous polyposis having duodenal adenomas, only 5% develop cancer. This risk rises to more than 30% in patients with more advanced disease in whom prophylactic surgery is often advised. Aims: The outcome of prophylactic pylorus preserving pancreatoduodenal resection performed for Spigelman Stage IV duodenal polyposis detected in an endoscopic surveillance programme is presented. Methods: FAP patients entered into the surveillance programme at St Mark's Hospital were included in this study. Endoscopy is performed with a side viewing duodenoscope to a set protocol. Data were collected prospectively on the Polyposis Register, and the case notes of patients undergoing prophylactic surgery reviewed retrospectively. Results: 419 FAP patients have entered the endoscopic surveillance programme since 1989. Between 1994 and 2002, 15 patients with advanced duodenal polyposis (six male, average age 54 years and 4 months) were referred for PPPDR. Six suffered major postoperative complications (40%). Although the pathology of the resected specimen revealed less severe changes in two patients, five with Stage IV disease showed adenocarcinoma, all ampullary. One patient (with benign histology) died from a pulmonary embolus shortly after hospital discharge. Four of the five patients with adenocarcinoma have died (10-36 months postoperatively) and one further patient has died form a brain tumour. The remainder are alive at a mean of 36 months (2-103). Conclusion: Surprise invasive adenocarcinoma was already present in 33% of patients. Survival once cancer has developed is poor. Prophylactic surgery may be most appropriate for individuals with large ampullary polyps. Advising resection for earlier stage disease is limited by the complication rate.
ACCESSION #
9747365

 

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