Jejunal cancer in patients with familial adenomatous polyposis.
OBJECTIVE
Familial adenomatous polyposis (FAP) is an inherited disease affecting approximately 1:10,000 newborns, characterized by the formation of numerous adenomas in the digestive tract. Surveillance and prophylactic treatment of colonic and duodenal manifestations of this disease have much influenced disease course and survival. In more recent years, it has become clear that adenoma formation in FAP patients is not restricted to the colon and duodenum. Accordingly, these adenomas might have malignant potential, although the actual risk is unknown.


METHODS
We report 3 cases of jejunal carcinoma in FAP patients and review data on incidence, prognosis, and risk factors of jejunoileal adenoma and carcinoma development in FAP.


RESULTS
Three patients with FAP aged 71, 57, and 59 years developed advanced duodenal adenomatosis and a jejunal carcinoma, which was associated with poor prognosis in 2 patients.


CONCLUSIONS
Jejunal adenomas in FAP patients are reported occasionally and can progress into adenocarcinoma with a poor prognosis. In the future a subset of FAP patients benefitting from jejunal surveillance should be identified.
Ruys
Anthony T
AT
Department of Surgery, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands.
Alderlieste
Yasser A
YA
Gouma
Dirk J
DJ
Dekker
Evelien
E
Mathus-Vliegen
Elisabeth M H
EM
D000230:Adenocarcinoma / Q000175:diagnosis* / Q000453:epidemiology; D000236:Adenoma / Q000175:diagnosis* / Q000453:epidemiology; D011125:Adenomatous Polyposis Coli / Q000150:complications* / Q000453:epidemiology; D000328:Adult; D006801:Humans; D015994:Incidence; D007580:Jejunal Neoplasms / Q000175:diagnosis* / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012307:Risk Factors