Angelina Jolie to remove ovaries: What are options for women at increased risk?

Angelina Jolie arriving at 69th Annual Golden Globe Awards at Beverly Hilton Hotel on January 15, 2012 in Beverly Hills Getty

Following her announcement that she underwent a double mastectomy, Angelina Jolie reportedly also plans to have surgery to remove her ovaries in order to lower her risk of developing ovarian cancer, People reported.

The actress' decision to proactively remove her breasts was chronicled in a New York Times editorial titled "My Medical Choice." Jolie explained that due to her mother's death from cancer at 56, she decided to undergo genetic testing. It was determined that she had a mutation of the BRCA1 gene, which increased her risk of developing breast cancer to 87 percent and her chance of getting ovarian cancer to 50 percent, Jolie wrote. In the general population, the chance of developing ovarian cancer during their lifetime is around 1.6 percent.

"Once I knew that this was my reality, I decided to be proactive and to minimize the risk as much I could," Jolie explained of her decision to have her breast tissues removed.

BRCA1 and BRCA2 are genes that everyone has that help with repairing DNA. Some people have mutated versions that increase their risk of getting certain types of cancer because their genes don't fix the damaged DNA. People with the BRCA1 mutation tend to develop cancer at an earlier age than the general population.

The inherited gene was first identified in the 1990s, Dr. Lynda Roman, co-director of the The Lynne Cohen Preventive Care Clinic for Women's Cancers at the University of Southern California, explained to CBSNews.com. Doctors normally recommend women go for genetic testing if they've had many relatives with breast or ovarian cancer, someone in their family who died of breast or ovarian cancer before the age of 80 or some member of their family who have had both breast and ovarian cancer.

Jolie's mother had breast cancer before she died from ovarian cancer, according to Pink Lotus Breast Center, the Beverly Hills clinic where she had her surgery. Her maternal grandmother was also diagnosed with ovarian cancer.

Dr. Pedro Escobar, assistant professor of surgery at the Cleveland Clinic Lerner College of Medicine at Case Western University, told CBSNews.com that previously it was thought that about 10 percent of the population had a mutated BRCA genes. As more research is published, the number may actually be higher as doctors discover that women with ovarian cancers with no familial history have the defective gene and certain insular populations actually have a higher incidence of the gene. Ashkenazi Jews, for example, may have an incidence rate of the genetic mutation as high as 1 in 40 people.

Typically, people with the BRCA mutations have a 20 to 40 percent chance of getting ovarian cancer, with people with the BRCA1 defect having a higher risk.

Women with this defective BRCA gene are often given the chance to radically reduce their chance of developing ovarian cancer by having their ovaries removed in a surgery known as an oophorectomy, in addition to having their fallopian tubes removed in a process called salpingectomy. Some women may choose to have a hysterectomy in which the uterus is removed, but because the BRCA1 gene isn't linked to as large of a risk increase for uterine cancer, some women do not choose to go forward with the more complex procedure.

Removal of the ovaries can reduce a woman's risk for ovarian cancer from 40 percent to less than 1 percent, Roman pointed out. Because the ovaries produce hormones, removing them also lowers the risk of developing some types of breast cancer as well.

"Risk-reducing surgery is the best way to decrease risk of ovarian cancer," Dr. Stephanie V. Blank, assistant professor in the division of gynecologic oncology in the department of obstetrics and gynecology at NYU-Langone Medical Center in New York, said to CBSNews.com.

Roman advises her patients with the defective BRCA 1 gene to get the procedure done before the age of 40 -- since women with the BRCA genes tend to get cancer younger -- or 10 years before the age a family member was diagnosed with breast or ovarian cancer. But, Blank pointed out that the decision is a personal choice influenced by many factors, so there is no "right" age to get the procedure done.

Blank added that unlike breast cancer, which has screening tests like mammograms available, no such test exists for ovarian cancer. It is normally discovered at a late stage, leaving doctors and patients with few treatment options. In 2013, the National Cancer Institute estimates that 22,240 will be diagnosed with ovarian cancer, and 14,030 women will die from it this year.

"Once (the cancer) has spread, it's quite deadly in the long run," Roman explained.

Surgery is not without risks. Removing the ovaries means that the woman will no longer be able to have children. Blank said that's why women who choose this step need to be sure that they have weighed these factors.

Women who have their ovaries removed also immediately go through menopause. They can experience everything from immediate bone loss, vaginal dryness, hot flashes and the inability to sleep.

These women can opt for hormone therapy in order to combat menopause. Women who just have their ovaries and fallopian tubes removed can just use estrogen, but women who choose to keep their uterus will also have to go on a progesterone regimen in order to thin their uterine lining to prevent endometrial (uterine lining) cancers.

However, women who have had a history of breast cancer may not be candidates for hormone therapy, because some breast cancers are fueled by hormones like estrogen. In general, Roman said in younger premenopausal patients, the positives of hormone replacement therapy are thought to outweigh the negatives so she always puts her patients on them. Eventually, as they reach their late 40s and early 50s, she weans them off the hormones as if they were naturally going through menopause.

"It's a little bit of a tricky question getting hormones after the natural age of 50," she explained. "It is a very different thing than getting it when you are young, when it would already be replacing what (would naturally) be there."

For women at risk for ovarian cancer who decide they don't want the surgery, there are other factors that can lower their chances of developing the disease. Taking oral birth control pills has been known to decrease the chance of getting ovarian cancer by up to 50 percent, all the doctors pointed out. Escobar added that having children also reduces the risk.

The connection between cancers and the BRCA mutations is still being researched. Recent studies by Dr. Robert Kurman, professor of gynecologic pathology at the Johns Hopkins University School of Medicine, have pinpointed the source of many ovarian cancers in people with the defective BRCA genes to lesions in the fallopian tubes, meaning what may have been thought of as "ovarian cancer" may actually tubular cancer. By studying the pathology of the removed fallopian tubes, doctors may find out more about what causes the cancers and find out ways to prevent it.

Escpbar also said we're learning more about modifiable factors that may reduce the risk for women with the BRCA mutations so women aren't helpless in their fight.

"You may find out that my genes are not repairing my DNA" and say "well, I'm going to be predisposed to a cancer. But, you can modify those risks," he said. "There are some lifestyle factors and genetic factors that can interfere. As we're finding out more things, the status will change."

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