BACKGROUND & AIMS: 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. Copyright 2010 AGA Institute. Published by Elsevier Inc. All rights reserved.
BACKGROUND & AIMS: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 FAPpatients 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 FAPpatients 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 FAPpatients are reported occasionally and can progress into adenocarcinoma with a poor prognosis. In the future a subset of FAPpatients benefitting from jejunal surveillance should be identified. Copyright 2010 AGA Institute. Published by Elsevier Inc. All rights reserved.
Authors: Noralane M Lindor; Tom C Smyrk; Sheila Buehler; Shanaka R Gunawardena; Brittany C Thomas; Paul Limburg; Salman Kirmani; Stephen N Thibodeau Journal: Fam Cancer Date: 2012-12 Impact factor: 2.375