Extraintestinal manifestations

Several other organs are involved in FAP but extra-intestinal manifestations rarely determine the clinical course of the disease.


Gastric adenomas do occur with increased frequency {425} but the most

Fig. 6.56 Diagram of stomach and duodenum showing the distribution of fundic gland polyps (open circles) and adenomas (solid circles) in FAP {425}. Adenomas are concentrated in the second part of the duodenum.

common abnormality is the fundic gland polyp. This is a non-neoplastic mucus retention type of polyp, grossly visible as a smooth dome-shaped nodule in the gastric body and fundus, usually multiple. Histologically, the lesion is characteristically undramatic, consisting of gastric body mucosa that is often normal apart from cystic dilatation of glands There is evidence of increased cell proliferation and dysplasia developing in these polyps {2144} but progression to adenocarcino-ma is only a rare occurrence {2214}.

Liver and biliary tract There is an increased incidence of hepa-toblastoma in the male infants of families with FAP {563; 578}. Dysplasia has been demonstrated in the bile duct and gallbladder epithelium in patients with FAP {1377} and these patients are at risk of developing adenocarcinoma of the biliary tree {1806}.

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