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Greetings, all. The Doctor is In.

Sad news today, I’m afraid. Last Tuesday, the New York Times published a letter from actress, director and WHO ambassador Angelina Jolie. Jolie wrote that she had undergone a double mastectomy (that is, removal of both breasts.) Following her mother’s the death at a young age from cancer, Jolie said that she had been genetically tested. She had discovered that she, too, also carried a particular mutation in her BRCA1 gene that raised her risk for breast and ovarian cancer enormously.

For most women, the risk of breast cancer is around 12%; but some mutations of the BRCA1 gene increase that risk. The degree of increased risk the mutation causes varies, depending on the type of mutation and how many mutated copies of the gene the woman carries. On average, a harmful BRCA1 mutation raises a woman’s risk for breast cancer to 60%; Jolie had been counselled that her risk was even higher at 87%. Harmful BRCA1 mutations are also associated with raising the risk of ovarian cancer from 1.4% to between 15 to 40%.

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Angelina Jolie

There is, however, no therapy currently available to fix this genetic mutation. It is not possible to shut down the faulty gene nor replace it with a non-harmful version. So, a woman carrying a BRCA1 mutation has a choice between two unsatisfactory options: live with the risk, have yearly MRI scans and mammograms, and hope that if a cancer begins, it is found soon enough for treatment to be successful.[1] Or, have one’s still  healthy breasts and ovaries removed as a preventive measure. Angelina Jolie chose the latter. She had her breasts removed. She also indicated in her letter to the New York Times that she may  later have her ovaries removed.

This choice is a horrific one to have to make—paying a pound of flesh to buy a reduced risk of the “dread disease”. In Jolie’s case, the poignancy of her choice is made even sharper by the nature of her most iconic film role: Lara Croft, Tomb Raider. This character (originally from a computer game) is distinguished by her long dark plait and her substantial, improbable bosom. (In playing the role, even Jolie’s glorious natural assets were slightly padded.) Jolie’s choice of a double mastectomy with subsequent cosmetic reconstruction is a brave one. In writing about it for other women, she has added another point to the list of reasons to admire her.

From a historical point of view, Jolie’s announcement is the latest example of a public figure speaking about her breast cancer. In 1973, Shirley Temple Black published an article in McCall’s magazine, saying how she had elected to undergo a two-step, simple mastectomy for her breast cancer. (Two step meaning two operations. In the first operation, some cells from the lump are removed and tested to see if they are cancerous. If the cells are cancerous, then—step two—the woman can opt for a second surgery to remove the tumour. In a simple mastectomy the breast is removed, but not the lymph nodes nor the underlying chest muscle.)

Shirley Temple Black

Shirley Temple Black

At the time, Temple Black’s choice for a two-step simple mastectomy was contentious. Most cancer surgeons had long favoured a more extensive operation: the radical mastectomy. In a radical mastectomy the lymph nodes and chest muscle are removed along with the breast itself. This more extensive operation was more likely to involve later complications of swelling, pain, and restricted motion. And the tradition was also to do it in one step—a single operation in which surgeons tested the tumour and removed it if it were cancerous. The patient would go under the anaesthetic not knowing whether or not she would wake up with a breast removed.

Shirley Temple Black’s surgeon had recommended the one-step, radical mastectomy option to her. But she didn’t want a one-step procedure. “The surgeon can make the incision,” she said, “I’ll make the decision.” And Temple Black had also heard of new research that had shown that the less invasive simple mastectomy had as good an outcome as the radical mastectomy. She insisted on a two-step procedure, and, when the biopsy results came back positive, a simple mastectomy. She wrote her McCall’s article to encourage other women to opt for two-step procedures and to push for more conservative surgery.

Happy Rockefeller

Happy Rockefeller

Betty Ford

Betty Ford

In 1974, Betty Ford, president Gerald Ford’s wife, announced through a press conference that she had undergone a one-step modified radical mastectomy. (Modified meaning surgeons left the chest muscle and only removed the breast and lymph nodes.) A few weeks later, Nelson Rockefeller also gave a press conference to say that his wife, Margaretta or “Happy”, had a two-step modified radical mastectomy.

Betty Rollin

Betty Rollin

And in 1975, Betty Rollin, an NBC news correspondent—who, ironically reported on the Ford and Rockefeller stories before discovering that she, too, had breast cancer—published First You Cry. The book in which Rollin talked about her experience being diagnosed with cancer, and her modified radical mastectomy, became a best-seller.

Together those four famous women raised awareness of breast cancer and the range of treatment options available. They also contributed to a trend away from the one-step radical mastectomy to two-step, more conservative procedures. This change also promoted women’s control over decisions about their cancer treatment and their bodies. Angelina Jolie has now joined their ranks. Her treatment—a preventive surgery based on genetic testing, is a more recent addition to breast cancer responses.

From ancient times, it had been clear that cancer sometimes ran in families. In the 1940s, systematic studies had shown that some rare cases of breast cancer—less than 10% of all breast cancer cases—followed family lines. This suggested there was some gene or genes involved in causing cancer in these cases. Developments in genetic screening and gene identification allowed a team of geneticists lead by Mary-Claire King at the University of California in Berkley to identify an area on chromosome 17 which seemed a likely location for this breast cancer gene. The particular gene involved was pinpointed in 1994 by geneticists at the University of Utah, and given the name BRCA1 (breast cancer susceptibility gene 1).

In most populations, the mutations in the BRCA1 gene that cause cancer are rare—only one in about 500 people carries a risk-raising mutation of their BRCA1 gene. (In Ashkenazi Jews the rate is higher—about one in 40—owing to the fact that Ashkenazi Jews share a small number of common ancestors, of whom one or more must have carried a BRCA1 mutation.) A second breast cancer gene, BRCA2, was identified in 1998. Other genes have also been found that are associated with hereditary breast cancer, but these are rarer than BRCA1 and 2 mutations.

From a historical point of view, Jolie’s announcement highlights these recent additions to the history of breast cancer: new technologies of genetic testing, new understanding of the inheritance of cancer, and surgery used preventively, rather than prophylacticly. But the announcement also highlights an essential sameness in the history of breast cancer: wrenching decisions and essentially unsatisfactory treatment options. Let us hope that when the next beat in this historical rhythm is played—when a public female figure speaks about her breast cancer in, say, 2030—she be announcing how the threat of cancer was successfully removed without surgery, without harsh side-effects and without on-going anxiety.

Till next time, stay well,

Dr Then

Interested? Want more?

Angelina Jolie’s letter to the NY Times:
Jolie, A, “My Medical Choice”, New York Times, http://www.nytimes.com/2013/05/14/opinion/my-medical-choice.html?ref=health, 14 May 2013.

Seminal papers on the discovery of the BRCA1 gene:
Hall JM, Lee MK,Newman B,Morrow JE,Anderson LA,Huey B, and King MC, “Linkage of early-onset familial breast cancer to chromosome 17q21”, Science 250 (1990):1684-9.

Miki Y, Swensen J, Shattuck-Eidens D, Futreal PA, Harshman K, Tavtigian S, Liu Q, Cochran C, Bennett LM, Ding W, et al, “A strong candidate for the breast and ovarian cancer susceptibility gene BRCA1”, Science. 1994 Oct 7;266(5182):66-71.

Summaries of current understanding of the BRCA genes:
National Cancer Institute. “BRCA1 and BRCA2: Cancer Risk and Genetic Testing.” National Institutes of Health, http://www.cancer.gov/cancertopics/factsheet/Risk/BRCA. 2009. Access date 2013.

Stanford Cancer Institute. “Hereditary Breast Ovarian Cancer Syndrome (BRCA1/BRCA2).” Stanford Medicine, http://cancer.stanford.edu/information/geneticsAndCancer/types/herbocs.html. 2013. Access date 2013.

On the history of breast cancer, and of famous women who spoke about their treatment:
Lerner, Barron H. The Breast Cancer Wars: hope, fear, and the pursuit of a cure in twentieth-century America. Oxford: Oxford University Press, 2001.

Olson, James S. Bathsheba’s Breast: Women, Cancer and History. Baltimore: Johns Hopkins University Press, 2005.

My own paper on breast cancer patient activists:
Dawes, Laura. “When Subjects Bite Back: The Bristol Cancer Help Centre Study and Increasing Consumer Involvement in UK Medical Research in the 1990s.” Social History of Medicine 25, no. 2 (2012): 500-19.

On the history of cancer in general:
Patterson, James T. The Dread Disease: Cancer and Modern American Culture. Cambridge: Harvard University Press, 1987.

[1] It may also be possible for the woman to take a drug such as tamoxifen or raloxifene to lower her risk of cancer. These drugs can have significant side effects, however, and the woman would still need to have regular scans.