Article

Jejunal Cancer in Patients With Familial Adenomatous Polyposis

Department of Surgery, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands.
Clinical gastroenterology and hepatology: the official clinical practice journal of the American Gastroenterological Association (Impact Factor: 7.9). 08/2010; 8(8):731-3. DOI: 10.1016/j.cgh.2010.04.008
Source: PubMed

ABSTRACT

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.
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.
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.
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.

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    • "The estimated lifetime risk for both patient groups is around 5%, which is more than 100-fold the risk in the general population [3] [4]. It is known that the risk of small bowel neoplasia in FAP is dependent upon the extent of duodenal polyposis [5], whereas relatively little is known about the prevalence and natural history of small bowel neoplasia in Lynch syndrome. Surveillance of the proximal small bowel is recommended in FAP but not (yet) in Lynch syndrome. "
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    ABSTRACT: Patients with familial adenomatous polyposis (FAP) and patients with Lynch syndrome have an increased risk of developing small intestinal neoplasia. In both conditions, the lifetime risk to develop small bowel cancer is estimated to be around 5%. In FAP, this risk is associated with the degree of duodenal polyposis, classically assessed by the Spigelman classification. For this reason, gastroduodenal surveillance with forward-viewing and side-viewing endoscopy is generally recommended. Studies using video capsule endoscopy and balloon-assisted enteroscopy in FAP patients have revealed that jejunal and ileal polyps occur frequently in FAP, especially in those with extensive duodenal polyposis. Nevertheless, the clinical relevance of small bowel polyps beyond the duodenum appears to be limited. Compared to FAP, little is known about the prevalence and natural history of small bowel neoplasia in Lynch syndrome. Surveillance of the small bowel is not recommended in Lynch syndrome, although recent data using capsule endoscopy provided promising results.
    Full-text · Article · Jun 2012 · Best practice & research. Clinical gastroenterology
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    No preview · Article · Aug 2010 · Clinical gastroenterology and hepatology: the official clinical practice journal of the American Gastroenterological Association
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    ABSTRACT: BACKGROUND & AIMS: Familial adenomatous polyposis (PAP) 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 mole recent years, it has become clear that adenoma formation in PAP 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 PAP patients and review data on incidence, prognosis, and risk factors of jejunoileal adenoma and carcinoma development in FAR RESULTS: Three patients with PAP 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
    No preview · Article · Oct 2010 · Clinical gastroenterology and hepatology: the official clinical practice journal of the American Gastroenterological Association
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