Women at risk for ovarian cancer face a complex decision-making process

Actress Angelina Jolie has ignited another worldwide conversation about cancer, revealing in the New York Times on Tuesday that she had undergone surgery, at age 39, to remove her ovaries and Fallopian tubes to prevent ovarian cancer. As she did 22 months ago, when she announced that she had had bilateral mastectomies to prevent breast cancer, Jolie cited her elevated risk of contracting the disease, the possible consequences and her desire to inform women of their options.

Jolie's op-ed won praise from oncologists and health experts for bringing a number of sensitive issues into the public discussion, but it also may have raised questions about the complex decision-making process faced by women at risk for ovarian cancer.

Here is some information that may help you evaluate the risk:

How common is ovarian cancer?

About 21,000 women will be diagnosed with ovarian cancer this year, according to Richard Wender, chief cancer control officer at the American Cancer Society, and 14,000 will die during that period. In the general population, one woman in 80 will develop ovarian cancer, said Jamie Bakkum-Gamez, a gynecologic oncologist at the Mayo Clinic in Rochester, Minn. The length of survival depends heavily on when the cancer is detected.

But Jolie is at much higher risk?

Yes. She carries a mutation of the BRCA1 gene most common in Eastern European Jews that gives her an 87 percent chance of developing breast cancer and a 50 percent chance of developing ovarian cancer, according to her article. Plus she has a long history of women in her family dying of cancer, including her mother, who was diagnosed with ovarian cancer at age 49. Women who carry a mutation of the BRCA2 also are at higher risk.

It's believed that less than 1 percent of American women carry the BRCA gene, Wender said, but there have been no good population studies in the United States.

But Jolie doesn't have cancer. Why did she have her ovaries removed?

The sad reality is that there is no reliable screening for ovarian cancer, experts say. Most ovarian cancers are discovered in the later stages of the tumor's progress, often after they have metastisized to other parts of the body, making treatment very difficult. Given her elevated risk of developing the disease, a risk benefit analysis clearly favors the surgery.

So is this some kind of special analysis available only to the wealthy and famous?

No. This is the current standard of care. "One of the central messages here," Wender said, "is that she followed the best medical advice. This is the same recommendation that would be given to any BRCA1 carrier who still has her ovaries. We have no good way to detect ovarian cancer."

What if she were younger or still wanted to have children?

Women facing this risk are encouraged to have children as soon as possible, said Marleen Meyers, a medical oncologist and director of the survivorship program NYU's Perlmutter Cancer Center in New York. If that's not possible, physicians may recommend frequent screenings involving ultrasound and tests for the CA125 protein, even though they are not as reliable as doctors would like.

What are the consequences of the surgery?

Jolie is now in very early menopause. She is taking hormones, though that practice is controversial, Meyers said, and some women who have had breast cancer cannot be put on hormones. As a result of the surgery, Jolie faces double the chance of dying from coronary artery disease before 80, according to this study, along with greater risk of osteoporosis, among other medical conditions. Also often overlooked is that all surgery, even laparoscopic procedures such as this one, carry some risk, including infection, Meyers said.

Menopause also brings issues such as hot flashes, night sweats, weight gain, thinning hair, skin changes and trouble sleeping.

Why so much praise for what Jolie did?

A couple of reasons. First, she reminded women facing this dilemma that there are preventive options and that they need to assess their risk by knowing their family cancer histories. "Women need to gather with their living relatives, both men and women, and they need to have a discussion about who in our family has had cancer," Wender said. "What kind of cancer did they have ? And . . . how old were they when they had it?"

Breast and ovarian cancers in the family are not the only ones to worry about, Meyers said. Colon, pancreatic, early prostate cancer, melanoma and male breast cancer in the family also can be a tipoff.

Meyers believes Jolie's efforts may also "bring menopause out of the closet," by prompting women to discuss their symptoms, and what can be done to relieve them, with their doctors. "It's going to take menopause and give it a whole new face and put it in a whole new light," she said. "It's a face of beauty. It's a face of courage."

What should you do if you're worried about ovarian cancer?

At the Mayo Clinic, any woman with a strong family history of these cancers is first sent to a genetic counselor, who will put together a risk profile and recommend testing, Bakkum-Gamez said. Carriers of the genetic mutations will undergo more frequent surveillance for breast and ovarian cancer, including an increased number of mammograms and MRIs. And they should discuss their options with their physicians, she said.

For women diagnosed with ovarian cancer, those choices include a form of chemotherapy approved in recent months by the Food and Drug Administration that exploits the BRCA gene's malfunction, she said.

Blood relatives, including men, should be informed of the diagnosis, so they, too, can assess their risks of cancer, she said.