The Complexity of Hereditary Cancer Syndromes

Counseling women at risk of ovarian and uterine cancer can present a challenge right from the start of making a genetic diagnosis, due to the overlapping of hereditary cancer syndromes. Ovarian cancer and uterine cancer are both associated with hereditary breast and colon cancer syndromes. It is quite common for women with a personal and/or family history of gynecologic cancers to present to the genetics clinic primarily concerned about gynecologic cancer, when in reality we usually have to open up the discussion to include other cancers for which the patient may not have expected to be at risk. It is vital that the clinician take cues from the patient and evaluate her understanding of this potentially expanded risk, while relying on other team members for support as well as surgical and screening risk management.

The article by Sue Rimes and her colleagues from MD Anderson Cancer Center was nicely done. The authors demonstrated how complex the relevant hereditary cancer syndromes can be and shared strategies employed at MD Anderson regarding communication with and management of high-risk patients. The approach taken in a National Cancer Institute (NCI)- designated Comprehensive Cancer Center is obviously widely multidisciplinary and resources utilized are different from those available in most community settings. Nevertheless, I believe that, to some degree, similar management can be offered in a community cancer genetics clinic, either through referral to local experts or through partnership with NCI-designated cancer center genetics clinics. I want to expand on sexual dysfunction, a specific problem faced by women at risk for gynecologic cancer who choose to have salpingo-oophorectomy.

DISCUSSING SEXUAL DYSFUNCTION

I found the authors’ comments about sexual dysfunction particularly valuable. Sexual dysfunction is a topic that many clinicians are not comfortable discussing. However, it is a critical point for our patients who will experience menopause as a result of salpingo-oophorectomy. It is recommended that counseling be initiated prior to surgery. Genetics professionals, physicians, nurses, and clinical social workers are all qualified to start sexual dysfunction discussions with our patients; unfortunately, we are not doing this very well.[1–2]

Premenopausal BRCA1 and BRCA2 mutation carriers who have prophylactic salpingo-oophorectomy do have some relief of sexual dysfunction with the use of hormone replacement therapy, but they do not return to their presurgical level of function.[3] While most women will still be satisfi ed with their decision to have surgery, some are not satisfi ed with the postsurgical outcome because prior to surgery they were not provided with sufficient information about what to expect. It is important for women to be informed of the possibility of sexual dysfunction and the ways in which it may (to some degree) be managed.

MD Anderson Cancer Center is fortunate to have a sexual therapist on staff who can further explore with patients and their partners the implications of sexual dysfunction. However, many community centers that do not have these experts on hand will need to have resources available to refer their patients to such experts for additional discussion, if necessary. Many cancer programs are starting survivorship programs that may include a sexual therapist. The cancer genetic counseling clinic can poise itself to be part of survivorship program on behalf of their “previvors.”

Counseling women at high risk for ovarian and uterine cancer is a complex process, from genetic diagnosis to the management of at-risk women. Rimes and colleagues have presented these challenging issues, and suggested ways to manage them, very well.

Financial Disclosure: The author has no significant fi nancial interest or other relationship with the manufacturers of any products or providers of any service mentioned in this article.