Two cases are added to the world literature of patients in whom an ade
nocarcinoma developed at the ileostomy site after total proctocolectom
y for ulcerative colitis. Fourteen additional cases have been reported
in the world literature; of these, 12 cases have been in patients wit
h ulcerative colitis, and four cases have been in patients with famili
al adenomatous polyposis. Adenocarcinoma of an ileostomy is not common
. However, in the analysis of the reported cases, patients with long-s
tanding ileostomies appear to be at a greater risk. With an aging ileo
stomy population, an increase in the number of cases may be seen. Thre
e hypotheses are discussed as potential causative pathways to this ent
ity. Continued analysis of these cases may yield information on the pa
thophysiology involved.