6

Breast Cancer

IN THIS CHAPTER YOU’LL DISCOVER

→ What Breast Cancer Is

→ Breast Cancer Statistics

→ Types of Breast Cancer

→ Genetics and Breast Cancer

→ Breast Cancer Staging

→ Signs and Symptoms

→ Specific Diagnostic Tests

→ Treatments and Types of Surgery

→ Breast Cancer in Men

SURVEYS SHOW THAT WOMEN consider breast cancer their biggest threat. This is not surprising, since breast cancer is among the most common of cancers — every woman personally knows someone who has had it. The disease carries the threat of not only death, but also disfigurement.

Time for a reality check! Breast cancer is indeed common, but if you get this form of cancer, you are more likely to survive it, as Nora Weinstein learned. Although she lost her grandmother and aunt to the disease and her mother battled it as well, today Weinstein is a breast cancer survivor. “There have been so many advances in the field of breast cancer that I feel very positive going forward,” says Weinstein, who was first diagnosed two decades ago and is now 70 years old.

What Is Breast Cancer?

Like other cancers, breast cancer begins when normal cells change and grow uncontrollably, forming a tumor. A tumor can be benign (noncancerous) or malignant (cancerous, with the possibility of spreading to other parts of the body). Some breast cancers are localized, meaning they are confined to the breast. When malignant, the cancer tends to spread to the bones, lungs, and liver, or, less often, to the brain.

DID YOU KNOW . . .

Although breast cancer occurs almost exclusively in women, it does occur in men. About 2,900 men in the United States develop breast cancer each year, and about 400 die from the disease. A man’s lifetime risk of developing breast cancer is one in every thousand.

Statistics

With the exclusion of skin cancer, breast cancer is the most common form of cancer in US women. An estimated 300,000 women will be diagnosed with breast cancer this year; this includes roughly 235,000 cases of invasive breast cancer (the type capable of spreading), and 65,000 of breast cancer in situ (from the Latin for “localized”), or the type that is not yet capable of spreading. About 40,000 American women die from the disease each year.

To better understand these statistics, the numbers show that about one in eight women will develop breast cancer, which is just under 12 percent of the population. This translates to about 125 new cases for every 10,000 women in the population.

After lung cancer, breast cancer is the second most common cause of cancer-related death in women. However, since 1990, the number of women who have died of breast cancer in the United States has steadily decreased, and today, 90 percent of those diagnosed are expected to survive for at least five years.

Most women who develop breast cancer are 60 years of age or older — a fact often overlooked because breast cancer victims as portrayed in the media tend to be much younger. However, young women are more likely to die from it.

About 20 percent of women with newly diagnosed breast cancer have a family history of the disease. This means having a first-degree relative (mother or sister), a second-degree relative (niece, grandparent, or half-sister), or a third-degree relative (first cousin or great-grandmother) who had breast cancer. Some genetic syndromes also raise the risk of ovarian cancer, so knowing your family history in that regard is important as well.

When you are assessing your genetic risk of breast cancer, don’t just focus on your maternal history (mother’s side of the family). You received half of your genes from your father, so the women on that side of the family count as well.

Outcome and Survival Rates

There are nearly three million breast cancer survivors in the United States.

Localized breast cancer, which is a small tumor(s) confined to one area of the breast, has a 98.6 percent cure rate. If the cancer has spread to another area within the breast, that survival rate drops to about 84.4 percent. But, as mentioned previously, even the nearly one-quarter of women with late-stage breast cancer survive five years or more.

“Today women are living longer as survivors of breast cancer. I have had the privilege to care for women with breast cancer for 30 years and have seen the overall cure rate increase from 55 percent to close to 90 percent,” says Dr. John Link, one of the world’s leading breast cancer oncologists and author of The Breast Cancer Survival Manual.

In addition, treatments today are less drastic than ever. Dr. Link continues: “I tell women that our top priority is to cure you, and that our secondary goal is to allow you to survive with the best quality of life and to return you to normalcy, and this is what happens in the vast majority of cases.”

DID YOU KNOW . . .

Women who develop breast cancer in one breast are at an increased risk of one to two percent per year for developing cancer in the opposite breast, even if they have no other breast cancer risk factors.

Types of Breast Cancer

There are many different ways of classifying breast cancer. The most common way used to be classifying it according to where the cancer occurred, and then determining how likely it was to spread. According to this classification system, most breast cancers fell into one of two types: ductal carcinoma, which occurs in the cells lining the breast ducts, and lobular carcinoma, which occurs in one or more of the breast’s 15 to 20 lobes.

You’ll still often find breast cancer classified this way, but genetic analysis has transformed the way it is staged and treated. This is why each breast tumor is subject to genetic testing. “It helps that a woman’s breast cancer be classified as one of four types, instead, which helps her and her doctors plan a more precise strategy,” says Dr. Link.

“Determining the type of cancer you have is critically important because your treatment course depends on it,” emphasizes Dr. Link. For instance, luminal A cancers can often be cured primarily with limited surgery and radiation, but chemotherapy is generally not needed. On the other hand, chemotherapy is a main treatment for luminal B cancers. “But even the aggressive cancers are yielding to new treatments,” he notes. “The HER2-type used to be the worst type of cancer, but now we have Herceptin and other drugs, which have raised the cure rate from 57 percent to the low 90s. We are thinking that eventually, it’s going to be curable,” Dr. Link adds.

LUMINAL A

This is a type of slow-growing, low-grade cancer, and the type most commonly discovered by mammogram screening. The outcomes are excellent, and the cure rate is more than 90 percent. These cancers all have estrogen and progesterone hormone receptors on the cell surface, which means they are easy to treat. They do have to be treated, though, because of the possibility that luminal A cells can change into luminal B cells.

LUMINAL B

These cells are more aggressive than luminal A. They are estrogen receptor positive, but often lose the progesterone receptor from the cancer cell surface. They also have the potential to spread to the lymph vessels.

TRIPLE NEGATIVE

Known also as “basal type” breast cancer, it is referred to as “triple negative” because the tumors don’t have estrogen or progesterone receptors, or evidence of the HER2-positive gene. These tumors account for 20 percent of all breast cancers, and they also tend to be fast growing, spreading, and aggressive. They are more common in younger women, and in those women who are carriers of the BRCA1 gene.

HER2-POSITIVE

About 20 percent of breast cancers overproduce a gene called the HER2 oncogene, which is a powerful, cancer-causing gene that sends messages to the cells to grow and spread.

Rare Breast Cancers Types

The four types of breast cancer cited above comprise the vast majority of breast cancer cases, but there are rare breast cancers as well. Of these many rare forms of breast cancer, two of the most common types are inflammatory breast cancer and Paget disease of the breast (or nipple).

INFLAMMATORY BREAST CANCER

IBC is an aggressive form of breast cancer, with symptoms that include a thickening and reddening of the skin over the breast. This aggressive, fast-spreading cancer, which was once almost always fatal, is now showing much better cure rates when treated aggressively with chemotherapy prior to surgery, Dr. Link says.

PAGET DISEASE OF THE BREAST OR NIPPLE

Named for 19th-century British doctor Sir James Paget, this is a rare cancer in the skin of the nipple or in the skin closely surrounding it. This form of the disease is usually, but not always, found with an underlying breast cancer. About one to three percent of breast cancers are Paget disease of the breast.

Genetics and Breast Cancer

Nancy Herrera was just turning 50 when she went for her routine mammogram. She was getting ready to go on a trip in a few days when she received the letter. “They said it was an abnormal reading, so they wanted me to come back,” she recalls. But, since Nancy had been religious about going for screenings, and there was no history of breast cancer in her family, she didn’t worry about it at all. But, on the second visit, the doctor told her they needed to do a biopsy. She underwent the procedure. “They told me I had ductal carcinoma in situ (DCIS). After that, everything went very fast.”

DCIS is really considered a pre-stage cancer finding, and sometimes it does not require treatment at all, only monitoring. But every case needs to be considered on an individual basis, and in Nancy’s case the diagnosis resulted in the removal of both of her breasts, and her ovaries as well. This is because Nancy was born with the BRCA1 gene mutation — a mutation that dramatically raised her risk of both breast and ovarian cancer.

The discovery of the BRCA1 and BRCA2 gene mutations in the 1990s transformed our knowledge of breast cancer. Women who carry the BRCA1 mutation, which is the primary one, account for only two percent of the population, yet they have a 70 percent increased lifetime risk of developing breast cancer. Not only that, but if they do develop breast cancer, it is far more likely to be triple negative — an aggressive and very difficult type to treat.

Nancy’s discovery that she was a BRCA1 carrier completely changed the picture of her breast cancer diagnosis. In fact, she’d been found to be a carrier years earlier when undergoing genetic testing during pregnancy, but the finding wasn’t pertinent at the time, so she had completely forgotten about it.

“The discussion went from ‘we can do a lumpectomy’ to ‘we are going to have to do a mastectomy and a full hysterectomy as well.’ I was shocked. I remember that first day when I was telling my family and I was crying so hard I couldn’t talk,” recalls Herrera.

Since then, she’s undergone the mastectomy and the hysterectomy as well as breast reconstruction, and she is looking forward to a healthy life “I’m healed. I still have to go for regular checkups, but I’m fine,” she says.

DID YOU KNOW . . .

The BRCA genetic mutations are not the only ones linked to increased breast cancer risk. There are additional more newly discovered genetic mutations, including one known as PALB2. The PALB2 gene is called a “partner” gene to BRCA because it works with it, but this is a far more rare mutation.

A decision to undergo a prophylactic, or preventive, mastectomy and hysterectomy is controversial, but not unusual, especially for BRCA1 carriers.

Unlike those diagnosed with breast cancer, there is a classification called previvors, which is the name given to women who are born with a strong hereditary risk of breast and ovarian cancer but haven’t yet developed the disease. Depending on their individual case, their options range from screening to tamoxifen therapy, or even prophylactic mastectomy. One famous previvor is Angelina Jolie, whose preventive mastectomy made headlines.

But with proper treatment, even women who have BRCA1 cancer do not necessarily face a shorter lifespan than their non-gene-mutation-carrying counterparts. A study of Polish breast cancer survivors published in the Journal of Clinical Oncology found that when the cancer was caught early, and especially if they underwent the preventive removal of their ovaries (to prevent estrogen from fueling the growth of the breast cancer), their 10-year survival rate was about 80 percent — virtually on par with those survivors not carrying the BRCA1 gene.

These are the clues that a genetic predisposition to breast cancer may exist in your family:

GOOD ADVICE

If you have breast cancer, get tested to find out if your form was inherited and, if so, inform your siblings and other relatives so they can assess their risk and determine if they should undergo genetic testing.

Stages of Breast Cancer

As with most cancers, the stage at which breast cancer is found predicates its treatment and its prognosis, but it is important to remember that there are survivors at every stage. Staging is also complex, especially in the case of breast cancer, with sub-stage classifications. The precise nature of the staging system illustrates the importance of administering the appropriate course of treatment from the start. Breast cancer staging, like other cancers, takes into account the presence of cancer cells, the size of the tumor (if any), and if/where it has spread.

STAGE 0, OR CANCER IN SITU

In this case, mutated cells are present but have not spread.

STAGE I

This stage indicates that a cancer has formed. Stage I cancers are subdivided into Stages IA and IB. In Stage IA, the tumor is two centimeters or smaller, and no cancer has spread outside the breast. In Stage IB, there may be no cancer, or a cancer two centimeters or smaller, but also tiny clusters of breast cancer cells are present in the lymph nodes.

STAGE II

This stage is divided into two sub-stages:

STAGE III

This stage is subdivided into three sub-stages:

STAGE IV

This is a situation in which the cancer has spread to other parts of the body, usually the bones, lungs, liver, or brain.

Signs and Symptoms of Breast Cancer

Special Diagnostic Tests

Here is a list of tests specifically performed to diagnose breast cancer. To learn more about general cancer testing, see chapter 2.

Clinical Breast Exam

The doctor will perform a physical examination and also examine the breasts for any sign of lumps, changes, or abnormalities. If a clinical breast exam uncovers an abnormality, one or more of the following imaging tests can be used as a diagnostic second step.

They include:

Mammogram

You are probably familiar with mammography as a screening test. A diagnostic mammogram is done to determine if a cancer is present, or to check out other potential breast cancer symptoms, such as breast changes or discharge. A clinical breast exam can also be done in conjunction with mammography.

Other Imaging Tests

Non-invasive ultrasound tests and magnetic resonance imaging (MRI) may be done to provide more information about the breast tumor and also determine if the cancer has spread.

Biopsy or Needle Aspiration

While other tests can suggest the presence of cancer, only a biopsy can confirm the diagnosis. In a biopsy, a small section of tissue is removed and examined under a microscope to see if cancerous cells are present.

Blood Tests and Tumor Markers

If a diagnosis of breast cancer is confirmed, several additional tests are performed. These include examining the tumor for various characteristics and to determine if any proteins, hormones, or other markers are present that can aid in the customization of treatment. For instance, breast cancer receptors are located in the cells of the tumor and the breast, and can influence the type of pharmacological, hormone, or other treatment given. These tests may include the following:

GOOD ADVICE

You should obtain a copy of your pathology report and have it explained to you in detail. This report contains all of the information that the pathologist observed when looking at cells, including the type, subtype, grade, and stage of your cancer, as well as the characteristics of any hormone receptors that were found. Such information is used to create a treatment plan.

Treatments

When most women think of breast cancer treatment, the term “radical mastectomy” comes to mind. This major form of surgery, which consisted of removing not only the breasts, but also the underlying chest muscles and associated lymph nodes, was the major treatment of breast cancer from 1895 to the mid-1970s, and even beyond. In fact, women would go under anesthesia for a breast biopsy and wake up to find their breasts gone. Such traumatic experiences have haunted generations of women.

Fortunately, clinical trials have finally proven that, in the case of breast cancer surgery, “more” is not necessarily better. “We used to over-treat breast cancer like crazy, but now we have much more of a handle on what treatment is appropriate, so our treatment is more targeted with less side effects,” says Dr. Link.

The importance of conservative treatment, or doing the least amount of surgery that will be effective, pertains not only to the breasts, but also to the lymph nodes. Lymph nodes are small structures that filter out harmful substances. They are arranged in a network of vessels and are the primary way that breast cancer spreads to other parts of the body. Until recently, clinical guidelines advised complete axillary node removal of all 20 to 30 lymph nodes if a woman’s sentinel biopsy was positive, no matter whether other lymph nodes were suspicious or not. (The sentinel node describes the first node to which the cancer is likely to spread.) Recently, though, a study in the Journal of the American Medical Association found that the results were just as good for women who had only the sentinel node removed, as opposed to the other nodes as well, as long as they were not affected. This is important, because the removal of lymph nodes can cause shoulder and arm symptoms including lymphedema, severe pain or numbness, and reduced range of motion.

Types of Surgery

MASTECTOMY

A mastectomy is the removal of one or two breasts. Although one or both breasts are removed, this surgery is less disfiguring than the radical mastectomy, with fewer side effects and quicker recovery. Mastectomies are done for the following reasons:

Women who have a mastectomy may wish to consider breast reconstruction, which is surgery to create a breast substitute. Reconstruction may be done with tissue from another part of the body or with synthetic implants. Reconstruction can be done at the same time as the mastectomy (immediate reconstruction) or at some point in the future (delayed reconstruction). This decision may depend on the characteristics of the particular cancer.

BREAST CONSERVING SURGERY

This form of surgery has replaced the radical mastectomy. Known also as local control, it allows for the removal of the cancer while leaving the breast in place.

The two major types of breast conserving surgery are lumpectomy, known also as wide local excision, or a partial mastectomy. For either to be performed, the cancer must be in the form of a small single tumor that can be removed completely. These surgeries are followed by radiation, which itself poses a risk of cancer and therefore is administered extremely conservatively, so the woman must never have undergone it before. A partial mastectomy is similar to a lumpectomy, but the size of the tumor can be larger.

Women who undergo a lumpectomy or partial mastectomy may consider a type of reconstruction known as oncoplastic surgery, which is done to match the breasts.

MASTECTOMY VS. LUMPECTOMY

Women with early-stage breast cancer will often receive a choice of whether to undergo a lumpectomy or opt for a mastectomy. On the surface, this choice might seem obvious, with most women opting to retain their breasts, but the choice is actually more complicated, Dr. Link says.

“There has been a huge increase of young women under age 40, 45, or 50 getting bilateral mastectomies who, in the past, would have gotten a lumpectomy with radiation. Part of this is due to the phenomenal results of plastic surgery with skin and nipple sparing. A lot of them are doing it because they don’t want to risk the cancer recurring, or they don’t want to go for periodic MRIs and biopsies.”

Because of such options, it’s best to take your time to make your decision. Seek out a second opinion, or even a third, so you’re confident you’ve made the right choice.

Other Modes of Therapy

CHEMOTHERAPY

Neoadjuvant chemotherapy is administered before surgery and is usually done to shrink early-stage breast cancer tumors so they can be removed during local, breast-sparing surgery. Adjuvant chemotherapy is administered after surgery. This is given to prevent the cancer from recurring. Chemotherapy is also used for cancers that have metastasized, or spread.

There is now an enormous array of chemotherapy drugs available, specific to the treatment of a variety of types of breast cancer. Research has also shown that certain drugs are more effective when given in combination, especially for adjuvant therapy.

IMMUNOTHERAPY

As opposed to chemotherapy, immunotherapy agents work by interfering with specific molecules to block the growth and spread of cancer. Such available agents for HER2-positive cancers are trastuzumab, pertuzmab, and lapitinib, though this is still an active research area.

HORMONAL THERAPY

This type of therapy is used to treat most tumors that test positive for either estrogen or progesterone receptors. The drugs effectively treat certain tumors by blocking the tumor’s receptors, cutting off the cancer cells’ fuel source, causing them to die. Tamoxifen, which blocks estrogen receptors, has proven revolutionary, not only in lowering the risk of recurrence in women who have had cancer, but also in preventing cancer in those at high risk of developing the disease.

Another type of hormonal therapy is the use of aromatase inhibitors (AIs). These decrease the amount of estrogen made by tissues other than the ovaries in postmenopausal women by blocking the aromatase enzyme. This enzyme changes weak male hormones, called androgens, into estrogen when the ovaries have stopped making estrogen during menopause. These drugs include anastrozole (Arimidex), letrozole (Femara), and exemestane (Aromasin).

RADIATION

Radiation therapy is typically given after a lumpectomy, and also following adjuvant chemotherapy, if recommended. In fact, modern surgery and radiation therapy are credited with reducing the risk that breast cancer will recur to less than five percent over the 10 years after treatment. This is the case whether the surgery was a lumpectomy or a mastectomy.

Adjuvant radiation therapy is also recommended for some women after a mastectomy. Whether or not radiation is warranted depends on the patient’s age, the size of the tumor, the number of lymph nodes found to contain cancer, whether the tumor was removed cleanly (clear margins), whether the tumor is hormone sensitive, if it is HER2 status, and other factors.

DID YOU KNOW . . .

Chemotherapy was once automatically administered following breast cancer surgery, but this is no longer the case because of the increased understanding of breast cancer, and the knowledge that some tumors respond to chemotherapy and some do not. Now, whether a woman should undergo chemotherapy is decided on a case-by-case basis.

Breast Cancer in Men

Robert Kaitz was a busy 53-year-old entrepreneur when he saw his doctor in 2006 for what he figured would be a simple office visit for a sore throat. Almost as an afterthought, he told the doctor he had a lump behind his nipple. “I figured it was a cyst, but the doctor told me you don’t get cysts there,” Kaitz recalls.

The diagnosis turned out to be advanced breast cancer, and within weeks, he was undergoing treatment. “I’m known for my sense of humor, so when people would ask, ‘What kind of cancer do you have?’ and I’d answer, ‘breast cancer,’ they’d pause and wait for the joke.”

There was no punch line — Kaitz indeed had breast cancer, and because the lump had been there for well over a year, the cancer had plenty of time to spread. As a result, he had to undergo a radical mastectomy, two rounds of chemotherapy and two rounds of radiation, before he was declared cancer-free.

“I was absolutely fortunate, but if I knew that men could get breast cancer, I would have gone to the doctor much sooner,” says Kaitz, especially since his mother is a breast cancer survivor. But, like most people, he simply assumed that men don’t develop breast cancer. Through rare, breast cancer more commonly occurs in men between 60 and 70 years old, but it may occur at any age.

“Some cancers are caught early, and the men do extremely well. If the cancer is caught later, it’s more difficult. It’s like any other breast cancer,” says Neil B. Friedman, MD, a breast cancer surgeon and director of the Hoffberger Breast Center at Mercy Medical Center in Baltimore, Maryland.

A recent study on breast cancer in men confirmed that survival rates are lower because their cancers tend to be diagnosed at a more advanced stage. And, as with women, the biggest known risk factor is genetic — BRCA 1 and BRCA 2 genes play the very same role in both genders.

Here are the other risk factors for breast cancer in men:

Breast cancer in men is treated generally the same as it is in women. Depending on the stage of the cancer, surgery and/or chemotherapy or radiation may be needed. Hormone therapy, especially the drug tamoxifen, is also used to treat male breast cancer.