Most polyps in FAP are sessile and spherical or lobulated. Scattered larger pedun-culated polyps are much less numerous {205; 835; 836; 688}. The colorectal polyps appear first in adolescence and, by the late teens, usually number thousands, typically carpeting the lining of the whole large bowel. Their number varies between families, in some being little more than 100, even in adults {1988}, whereas, in the majority of families, there are profuse polyps, numbering thousands. Typically, the polyps are scattered evenly along the whole large bowel but, in over one third of cases, their density is greatest in the proximal colon. Adult patients with rectal sparing have been described, even when adenocarcinoma was present in the right colon {1503}. In any one patient the polyps range from barely visible mucosal nodules to pedunculated polyps of up to 1 cm or more. In some patients and families the adeno-

Fig. 6.54 Small ulcerated adenocarcinoma with rolled edges (arrowhead), accompanied by numerous adenomas in a patient with FAP. Polypectomy scars are present.

mas mostly measure only a few millimetres while in others they are larger, with polyps up to several centimetres. In contrast, in attenuated FAP, the polyps are so few that they may not be noticed at rigid sigmoidoscopy. Polyps rarely appear until late childhood {216} and are rarely larger than 1 cm until adulthood. Adenocarcinomas arise in only a small percentage of the adenomas.

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