Small bowel cancer is one of the tumors associated with Lynch syndrome.
To advise on screening for this tumor, it is paramount to be informed about the lifetime risk.
Dr ten Kate and colleagues from the Netherlands calculated the lifetime risk of small bowel cancer in Lynch syndrome and identified possible risk factors.
Clinical and pathological data were collected on 1496 proven or putative carriers of a mismatch repair gene mutation from 189 families.
Kaplan-Meier survival analysis was used to calculate the lifetime risk, and to assess potential risk factors.
The researchers identified 2% of 1496 putative mutation carriers with small bowel cancer.
|The lifetime risk of developing small bowel cancer was 4%|
The median age at diagnosis was 52 years.
The lifetime risk of developing small bowel cancer was 4%.
The team found there was no difference in risk between males and females, or between MLH1 and MSH2 mutation carriers.
The research team did not observe small bowel cancer in MSH6 mutation carriers.
The previous occurrence of colorectal cancer and a family history of small bowel cancer did not increase the risk significantly.
Dr ten Kate's team concluded, "Approximately, 1 out of 25 mutation carriers will develop small bowel cancer during life."
"No specific risk factors were identified."
"The risk appeared to be too low to advise screening by means of an invasive burdensome procedure like double balloon enteroscopy."
"However, screening by a non-invasive procedure might be considered if future studies will show its cost effectiveness."
"In patients with unexplained abdominal complaints and/or unexplained iron deficiency anemia, small bowel cancer should be considered."