Actress Angelina Jolie and her daughters Vivienne and Shiloh attending the 2015 Kids’ Choice Awards in Los Angeles, California, on Saturday. Photo: Mario Anzuoni/ReutersThe “faulty” gene which prompted Hollywood actress Angelina Jolie to have a preventive double mastectomy and, more recently, to remove her ovaries and fallopian tubes is very rare in Malta, according to breast surgeon Joseph Debono.
Writing in The New York Times, Ms Jolie said that her doctors agreed that surgery to remove her tubes and ovaries was the best option because, on top of the BRCA 1 gene mutation, her grandmother, mother and aunt had all died of cancer.
Two years ago, she ignited a worldwide discussion about options for women at high risk for breast cancer when she wrote that she had had both breasts removed because BRCA1 increased her risk of breast cancer.
“Interestingly, the BRCA1 and BRCA2 genes are very rare among the Maltese,” Mr Debono explained.
“The incidence is much higher in Nordic countries, such as Finland and Iceland. In fact, the majority of cases in Malta involve foreigners.”
Research was currently being undertaken to possibly identify other genetic mutations, other than BRCA1 and BRCA2, which could result in cancer among the Maltese population.
To his knowledge, no Maltese woman has yet opted to have a preventive mastectomy or oophorectomy (surgery removing the ovaries).
“The closest case we had was when a patient decided to remove her breasts while she was undergoing surgery to remove tumours.”
Every case is unique
The selection process for such surgeries is a very rigid one – there are specific, recognised criteria which focus on a person’s family history of cancer.
“Such surgeries are called ‘risk reducing surgeries’ because they do not completely eliminate the risk of cancer.”
Women identified as high risk would consequently be referred to geneticists.
There were psychological factors to be considered in making such a decision, Mr Debono pointed out, as well as a number of side-effects such as the removal of a woman’s ovaries putting her into forced menopause.
Geneticist Christian Scerri explained that the level of risk was determined through a person’s family history, age and through genetic testing. “Every case is unique. For instance removing the ovaries of a woman aged under 30 can lead to osteoporosis.”
A person’s family doctor could provide advice based on the family history, and through looking at whether the patient has at least one relative who developed cancer below the age of 40 or more than one relative below the age of 50.
Malta, Prof. Scerri continued, followed the breast cancer guidelines issued by the UK’s National Institute for Health and Care Excellence (NICE).
In contrast with the United States, these guidelines do not recommend preventive mastectomy or oophorectomy for women at high risk of cancer, although women are free to take that option.
Preventive screening is instead recommended according to different cases. Women at moderate risk are advised to undergo a mammogram every year once they turn 40.
Women deemed to be at high risk and who have the gene mutation are advised to undergo annual MRIs if they are aged between 20 and 30.
If they are considered to be at high risk but do not carry the gene, they are advised to undergo a yearly MRI between the ages of 30 and 40 and a yearly mammogram after turning 40.
Prof. Scerri’s ultimate advice is that when in doubt, consult your family doctor.
Speaking to this newspaper, breast cancer survivor Carmen Hassanien said she would not think twice about having her remaining breast and ovaries removed if her doctors determined her to be at high risk.
Two and a half years ago, Ms Hassanien battled breast cancer at the age of 35, undergoing chemotherapy, radiology and, finally, a mastectomy.
“In fact, when I was having the mastectomy, I told the surgeons that they could remove the other breast as well but they told me that it was not necessary.
“I would not think twice about undergoing preventive surgery – it’s infinitely better than the agony of chemotherapy or of living in fear of developing cancer.”