ASCO: Lynch Syndrome: A Multitude of Predispositions (note ovarian and breast cancer risk %)

The ASCO Post

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(Lynch Syndrome in women)

"In addition to endometrial cancer, cancers that occur in excess in germline mutation carriers for Lynch syndrome are the aforementioned colon and ovary cancers; cancers of the stomach, small bowel, pancreas, upper uroepithelial tract (ureter and renal pelvis), breast, and prostate; sebaceous adenomas, sebaceous carcinomas, multiple keratoacanthomas, and possibly other skin cancers in the Muir-Torre syndrome variant; and glioblastoma multiforme in the Turcot syndrome variant."
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"The current uncertainty regarding the relative frequencies of cancers of various anatomic sites in Lynch syndrome poses a difficulty in commenting on the syndrome’s overall cancer spectrum. It is even more vexing to address the order in which these cancers are prone to occur. What we do know is that there is an enormous lifetime risk of cancer at multiple anatomic sites in Lynch syndrome....... Lynch syndrome has become known to be the most frequent hereditary colorectal cancer–prone disorder, but is also now known to predispose to cancers of an almost unlimited number of other sites.....

"In addition, compared with the general population, women carrying a mismatch repair gene mutation have a 19-fold greater risk of developing ovarian cancer and a 4-fold increased risk of developing breast cancer.<sup>5"

"Given the excess of gynecologic cancer (endometrium and ovary) in Lynch syndrome and the respective limitations of their screening, which has virtually no benefit for ovarian cancer and is of only limited use in endometrial cancer, in our opinion a better choice is the option for prophylactic hysterectomy and bilateral salpingo-oophorectomy, once family is completed and the patient is fully accepting."</sup>