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. 2002 May;50(5):636-41.
doi: 10.1136/gut.50.5.636.

Duodenal cancer in patients with familial adenomatous polyposis (FAP): results of a 10 year prospective study

C J Groves  1 , B P Saunders, A D Spigelman, R K S Phillips
Affiliations

Duodenal cancer in patients with familial adenomatous polyposis (FAP): results of a 10 year prospective study

C J Groves et al. Gut. 2002 May.

Abstract

Background: Duodenal cancer is one of the leading causes of death in familial adenomatous polyposis (FAP) patients. An endoscopic surveillance programme was therefore initiated in 1988, the outcome of which is described in this paper.

Methods: We report the 10 year follow up of 114 patients with FAP who were prospectively screened for the presence and severity of duodenal adenomas.

Results: Six of 114 patients (median age 67 years) developed duodenal adenocarcinoma. Four of these were from 11 patients who originally had Spigelman stage IV disease (advanced duodenal polyposis), which gives a 36% risk within this group of developing cancer. One case of duodenal cancer arose from 41 patients who originally had stage III disease (2%) and one cancer arose from 44 patients with original stage II disease (2%). All six patients have died: five were inoperable and one had recurrence three years after a pancreaticoduodenectomy. There was no association between duodenal cancer and site of germline mutation of the APC gene.

Conclusions: Surveillance for duodenal adenocarcinoma and subsequent early referral for curative surgery has not been effective. Selection of patients with advanced but benign (Spigelman stage IV) duodenal polyposis for prophylactic pancreaticoduodenectomy should therefore be considered and can now be justified on the basis of these results. More comprehensive endoscopic surveillance of high risk (stage III and IV) patients is needed in an attempt to avoid underestimating the severity of duodenal polyposis, and to evaluate the role of endoscopic therapy in preventing advanced disease.

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Figures

Figure 1
Figure 1
Position of germline mutation on the APC gene for patients with stage 0, I, or II duodenal polyposis versus stage III or IV polyposis or duodenal cancer. Where there was more than one family per mutation, individuals were grouped into families, seen at C694T and at codons 1061 and 1309.
Figure 2
Figure 2
Algorithm for the management of duodenal polyposis in familial adenomatous polyposis. CP, chemoprevention; ET, endoscopic therapy; GA, general anaesthetic; PPPD, pancreas (and pylorus) preserving pancreaticoduodenectomy.
See this image and copyright information in PMC

References

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