Surveillance and management of upper gastrointestinal disease in Familial Adenomatous Polyposis
Michelle C. Gallagher1, Robin K.S. Phillips1 and Steffen Bulow2 1The Polyposis Registry; Cancer Research UK Colorectal Cancer Unit, St Mark’s Hospital, Northwick Park, Watford Road, HA1 3UJ, Harrow, UK; 2The Danish Polyposis Registry, Hvidovre University Hospital, DK 2650, Hvidovre, DenmarkI
ntroduction With the exception of the oesophagus, the entire mucosa of the upper gastrointestinal tract appears to be at
increased risk from neoplasia in patients affected by the Mendelean dominant family cancer syndrome Familial Adenomatous Polyposis (FAP) [1, 2]. Gastric polyps were first described in 1895 [3] and duodenal polyps in 1904 [4], and the earliest published report of FAPassociated duodenal carcinoma appearing in 1935 [5]. Cases were reported sporadically until the 1970s [6–9], but it was not until the later part of the 20th century that the clinical importance of foregut disease, particularly duodenal adenomatosis, was more widely recognised [1, 10, 11] and attention focussed on the surveillance and management of the upper gastrointestinal tract. The purpose of this review is to summarise current knowledge regarding gastroduodenal disease in FAP and to examine the potential treatment options for affected individuals.
Epidemiology The upper gastrointestinal tract, including the duodenum, pancreas and hepatobiliary system is the commonest
extra-colonic site of malignancy in FAP [1, 10, 12] and, together with desmoid disease, duodenal and periampullary cancer, constitutes the second most common cause of death in screened populations [1, 11, 13, 14]. Whilst duodenal adenomas and carcinoma are rare
in the general population (average age of onset in the mid 60s, male predominance, incidence between 0.01 and 0.04% [15–17]], the majority of FAP patients are likely to develop duodenal involvement with a lifetime cancer risk approaching 5% [18, 19]. The average age at cancer diagnosis, 52 years (range 18–78), is earlier than in sporadic cases [1, 9, 20], and the relative risk of duodenal and periampullary carcinoma in FAP is estimated to be between 100 and 330 times that for the general population [1, 11, 21, 22].
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