“Fortunately, there’s been a lot of work for patients with Lynch syndrome,” says Dr. Jones. “It used to be that they had really aggressive cancers, and we just didn’t have a great way of treating them. But over the last five to seven years, we’ve had an explosion of drugs called immunotherapies. We’ve seen dramatic responses and unlikely cures in patients.”
The lifetime risk of each cancer in people with Lynch syndrome is gene-specific and may be modified by environmental factors.
Furthermore, the benefits of surveillance strategies need to be balanced against the risk of over-diagnosis and be supported by evidence of improved outcomes from cancer diagnosis in surveillance.
Therefore, people with Lynch syndrome may benefit from a personalised management approach.
FORCE(USA) has compiled resources and information to help people make informed decisions and receive support.
People diagnosed with Lynch syndrome have an increased risk for colorectal, endometrial and other cancers. Options are available for cancer treatment, screening and prevention for people with Lynch syndrome.
Regular bowel screening and aspirin reduce colorectal cancer among patients with LS but extracolonic cancers are difficult to detect and manage. This study suggests that RS reduces morbidity associated with extracolonic cancers.
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Bowel cancer deaths can be prevented through the early detection and removal of polyps.
“We want to prevent polyps turning into early-stage cancers,” Bowel Cancer Australia Medical Director, Graham Newstead AM said.
The recommendations from the EHTG and ESCP for identification of patients with Lynch syndrome, colorectal surveillance, surgical management of colorectal cancer, lifestyle and chemoprevention in Lynch syndrome that reached a consensus (at least 80 per cent) are presented.
Cumulative Lifetime risks of cancer in Lynch Syndrome
Endometrial: Up to 57%
Ovarian: Up to 17%
Identifying patients with Lynch syndrome is clinically important because these patients have up to 80 percent lifetime risk of colorectal cancer and up to 60 percent lifetime risk of endometrial cancer.
These patients also have an increased risk for other primary cancers including gastric, ovarian, small bowel, urothelial (ureter, renal pelvis), biliary tract, pancreatic, brain (glioblastoma), sebaceous gland adenomas, and keratoacanthomas.
Individuals found to have a deleterious Lynch syndrome mutation are at increased cancer risk with the greatest risk of colorectal and endometrial cancers, followed by gastric and ovarian cancers. Fortunately, there are risk management guidelines for carriers of Lynch syndrome which are associated with a decrease in cancer-related deaths.
Management guidelines for Lynch syndrome may require revision in light of these different gene and gender-specific risks and the good prognosis for the most commonly associated cancers.