In the wake of Angelina Jolie Pitt’s announcement that she had her ovaries and fallopian tubes removed to reduce her risk of ovarian cancer doctors are urging women to proceed cautiously before following her example. She shared her story in an op–ed piece published in The New York Times on Tuesday.



The procedure makes sense for relatively few women, says Noah Kauff, director of Ovarian Cancer Screening and Prevention at Memorial Sloan Kettering Cancer Center. He and others say it is worth considering mainly for those who have inherited a particular genetic mutation known by their associated genes, BRCA1 and BRCA2, particularly if they have a strong family history of breast or ovarian cancer. Jolie Pitt has both. She inherited the BRCA1 mutation, and her mother, grandmother and aunt all died of cancer.



The recent operation is her second surgery to avoid cancer. In May 2013 she reported that she had undergone a double mastectomy to prevent breast cancer. She had both the mastectomies and the newest procedure in part because BRCA1 elevates cancer risk substantially. For women with the mutation, the chance of developing breast cancer can be as high as 65 percent. In contrast, the risk for the general population is 12 percent, according to the National Cancer Institute. The mutation can also increase ovarian cancer risk to 39 percent whereas it is only 1.4 percent for the general population. BRCA2 gene mutations can increase risk similarly. BRCA genes code for tumor-suppressor proteins; when they have deleterious mutations, cancers can arise more readily.



But only 1 percent of women carry one of these mutations. For that reason, the U.S. Preventive Services Task Force recommends (pdf) that only women with a family history of cancer receive a genetic test for the mutations, which can cost several hundred to several thousand dollars depending on insurance coverage. Public health experts suggest that women consult their doctors to understand their personal risk. What they do not want to see is a repeat of what happened after Jolie had her mastectomies: Many women rushed out to get genetic tests and the procedure, when relatively few of them needed to do either. Three quarters of Americans knew of Jolie Pitt's story, a study found, but fewer than 10 percent could accurately identify how her risk compared with that of women without her mutation.



Some women, like Jolie Pitt, end up having to resort to extreme measures because waiting until ovarian cancer is detected can be too late. The disease does not have obvious symptoms and current detection methods do not work very well. Of the more than 21,000 women who are diagnosed with ovarian cancer every year, more than 14,000 die from it. Screening, through transvaginal ultrasonography and CA-125 protein testing (which Jolie Pitt says she received), is so unreliable that the task force even recommends against them unless women are at high risk. Meanwhile signs of the disease resemble symptoms that women commonly experience during menstruation or other less serious afflictions, such as stomach pain, vaginal bleeding, weight gain or loss, gas, nausea and a heavy feeling in the pelvis.





Credit: National Cancer Institute



For women with a BRCA mutation, preventative surgery can be a lifesaving solution. One study found that women with the mutation who had salpingo-oophorectomy—where the fallopian tubes and ovaries are removed—reduced their risk of getting ovarian cancer by 80 percent and their risk of getting breast cancer by half. Removing the ovaries reduces breast cancer risk as well because they secrete estrogen, which can promote the growth of breast cancer cells.



In a salpingo-oophorectomy (pdf) small incisions are made in the stomach, and a thin camera guides the removal of the ovaries and fallopian tubes with a surgical tool. The patient is under general anesthesia during the procedure and can make a full recovery within a couple weeks. Although the surgery itself is rather simple, its effects can be far-reaching. Removing the ovaries triggers menopause and all of the symptoms that come with it. For women under 45, removing the ovaries can also result in bone thinning and increased risk of heart disease because of reduced estrogen levels. Women who have the procedure often have to undergo hormone therapy to reduce these effects. And although having a salpingo-oophorectomy does reduce the risk of developing cancer, it does not remove it completely. Fortunately, nearly all insurance companies cover the full cost of risk-reducing surgery if doctors recommend it, Kauff says.



Other options to prevent ovarian cancer besides salpingo-oophorectomy include taking birth control pills, which can reduce the risk for all women by half after five years of use. Women could also have just the fallopian tubes removed and leave their ovaries, which Jolie Pitt mentioned in her article, in order to postpone menopause. Kauff says there is some evidence suggesting a proportion of ovarian cancers actually originate in cells from the fallopian tube, so just removing them could be an effective prevention strategy but there is not enough data to know for sure yet. Karen Lu, chair of the Department of Gynecologic Oncology and Reproductive Medicine at The University of Texas M. D. Anderson Cancer Center, says she considers removing only the fallopian tubes as an intermediary step to ward off menopause before later removing the ovaries, and recommends it only as part of a clinical trial.



Kauff says there is still some misinformation on the issues, such as the recommended timing of the surgery. (Jolie Pitt said 10 years before the age her mother was at diagnosis but doctors say it should occur by age 40 and when childbearing is complete.) He adds, however, that her announcement is helpful in encouraging more women to talk about cancer risk. "She is causing discussion of this issue," he says. "And then, hopefully, women will go to their gynecologists and say, 'What can we do to reduce risk? What can't we do to reduce risk?'"