Jejunal cancer in patients with familial adenomatous polyposis
Anthony T Ruys1, Yasser A Alderlieste, Dirk J Gouma
1Department of Surgery, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands.
Insights
Familial adenomatous polyposis (FAP) patients can develop jejunal (small intestine) cancers, which are associated with a poor prognosis. Further research is needed to identify FAP patients who may benefit from jejunal surveillance.
Area of Science:
- Gastroenterology
- Oncology
- Genetics
Background:
- Familial adenomatous polyposis (FAP) is an inherited condition affecting approximately 1 in 10,000 newborns, characterized by numerous digestive tract adenomas.
- While colonic and duodenal manifestations are well-studied, adenoma formation in FAP is now recognized to occur in other areas of the digestive tract.
- The malignant potential of these non-colonic, non-duodenal adenomas remains largely unknown.
Observation:
- This study reports three cases of jejunal carcinoma in FAP patients, aged 71, 57, and 59.
- All three patients presented with advanced duodenal adenomatosis.
- Two of the three patients experienced a poor prognosis due to their jejunal carcinoma.
Findings:
- Jejunal adenomas, though occasionally reported in FAP, can progress to adenocarcinoma.
- Jejunal adenocarcinoma in FAP patients is associated with a poor prognosis, as evidenced by the cases presented.
- The incidence, prognosis, and risk factors for jejunoileal adenoma and carcinoma in FAP require further investigation.
Implications:
- The findings suggest that jejunal surveillance may be beneficial for a subset of FAP patients.
- Identifying specific risk factors and patient subgroups for jejunal surveillance is crucial for improving outcomes.
- This research highlights the need to consider the entire digestive tract when managing FAP patients.
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.
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