Breast - Inflammatory CancerLast Updated: October 30, 2009 This section has been reviewed and approved by the Cancer.Net Editorial Board, 10/08 Overview
Inflammatory breast cancer is a rare form of breast cancer. The cancer gets its name because the symptoms are like those of mastitis (inflammation of the breast) and include redness, tenderness, swelling, and pain in the breast. However, unlike mastitis, inflammatory breast cancer does not improve with antibiotic treatment. The breast is mainly composed of fatty tissue. Within this tissue is a network of lobes, which are made up of tiny, tube-like structures called lobules that contain milk glands. Tiny ducts connect the glands, lobules, and lobes, carrying the milk from the lobes to the nipple, located in the middle of the areola (darker area that surrounds the nipple of the breast). Blood and lymph vessels run throughout the breast; blood nourishes the cells, and the lymph system drains bodily waste products. The lymph vessels connect to lymph nodes, which are tiny, bean-shaped organs that help fight infection. Cancer may begin as a single, genetically abnormal cell. As this one cell divides, it eventually becomes a tumor (a mass of cells) and develops a blood supply to nourish its continued growth. At some point, cells may break off from the primary mass and move to other parts of the body in a process called metastasis. In inflammatory breast cancer, the cancer cells block the lymph vessels within the breast. Because this type of breast cancer can grow quickly, it is treated with a combination of chemotherapy, surgery, radiation therapy, and hormone therapy. Statistics Estimates of the incidence of inflammatory breast cancer range from 1% to 5% of all breast cancers. Because inflammatory breast cancer can grow and spread quickly, it may be advanced at the time of diagnosis and may be treated similarly to a late-stage breast cancer. The five-year relative survival rate (percentage of people who survive at least five years after the cancer is detected, excluding those who die from other diseases ) of people with inflammatory breast cancer is between 25% and 50%. Cancer survival statistics should be interpreted with caution. These estimates are based on data from thousands of cases of this type of cancer in the United States each year, but the actual risk for a particular individual may differ. It is not possible to tell a person how long he or she will live with inflammatory breast cancer. Because survival statistics are measured in five-year intervals, they may not represent advances made in the treatment or diagnosis of this cancer. Source: National Cancer Institute Find out more about basic cancer terms used in this section. Medical Illustrations Risk Factors
A risk factor is anything that increases a person’s chance of developing cancer. Some risk factors can be controlled, such as smoking, and some cannot be controlled, such as age and family history. Although risk factors can influence the development of cancer, most directly do not cause cancer. Some people with several risk factors never develop cancer, while others with no known risk factors do. However, knowing your risk factors and communicating them to your doctor can help you make more informed lifestyle and health-care choices. It is not known what factors can raise a person’s risk of inflammatory breast cancer. A family history of inflammatory breast cancer may increase the risk of developing the disease, but to date, no gene mutation has been found specifically for this type of breast cance Symptoms
Women with inflammatory breast cancer may experience the following symptoms. Sometimes, women with inflammatory breast cancer do not show any of these symptoms. Or, these symptoms may be caused by a medical condition that is not cancer. If you are concerned about a symptom on this list, please talk with your doctor. Symptoms of inflammatory breast cancer may appear quickly and within a short time of each other.
Diagnosis
Doctors use many tests to diagnose cancer and determine if it has metastasized (spread). Some tests may also determine which treatments may be the most effective. For most types of cancer, a biopsy is the only way to make a definitive diagnosis of cancer. If a biopsy is not possible, the doctor may suggest other tests that will help make a diagnosis. Imaging tests may be used to find out whether the cancer has metastasized. Your doctor may consider these factors when choosing a diagnostic test:
In addition to a physical examination, the following tests may be used to diagnose inflammatory breast cancer: Imaging tests Diagnostic mammography. Diagnostic mammography is similar to screening mammography (x-ray of the breast) except that more views (pictures) of the breast are taken, and it is often used when a woman is experiencing signs, such as nipple discharge or a new lump. Diagnostic mammography may also be used if something suspicious is found on a screening mammogram.Ultrasound. An ultrasound uses high-frequency sound waves to create an image of the breast tissue. An ultrasound may distinguish between a solid mass, which may be cancer, and a fluid-filled cyst, which is usually not cancer. Magnetic resonance imaging (MRI). An MRI uses magnetic fields, not x-rays, to produce detailed images of the body. A contrast medium (a special dye) may be injected into a patient’s vein to create a clearer picture. An MRI may be used once a woman has been diagnosed with cancer to check the other breast for cancer, but the benefit of this is controversial. It may also be used for screening. According to the American Cancer Society, women at high risk for breast cancer (for example, women with breast cancer [BRCA] gene mutations or a strong family history of breast cancer) should receive MRI screening along with a mammogram. MRI may often be better at seeing a small mass within a woman’s breast than a mammogram or ultrasound, especially for women with very dense breast tissue, but has a higher rate of false-positive test results (a test result that indicates cancer when there is no cancer present) and may result in more biopsies. In addition, an MRI does not show calcifications, which could indicate in situ breast cancer. Talk with your doctor for more information. Surgical tests Biopsy. A biopsy is the removal of a small amount of tissue for examination under a microscope. Other tests can suggest that cancer is present, but only a biopsy can make a definite diagnosis. The sample removed from the biopsy is analyzed by a pathologist (a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease).
If cancer is diagnosed, a second surgery may be needed to get a clear margin (area of tissue around the tumor where there are no cancer cells) and/or remove lymph nodes. Doctors may also test the tissue from a biopsy to help guide treatment decisions. The tests include:
Blood tests The doctor may also need to do blood tests to learn more about the cancer. Complete blood count (CBC). CBC is a blood test done to determine the following:
Alkaline phosphatase levels. High levels of this enzyme could indicate the disease has spread to the liver, bone, or bile ducts. Total bilirubin count, serum glutamic-oxaloacetic transaminase(SGOT), and serum glutamate pyruvate transaminase (SGPT) levels. These tests evaluate liver function. High levels of any of these substances can indicate liver damage, a signal of possible spread to that organ. Tumor marker tests. A tumor marker (also called a serum marker or biomarker) is a substance found in a person's blood, urine, or body tissue. The presence of a tumor marker, or having higher or lower than normal levels of a tumor marker, may indicate an abnormal process in the body, which could be because of cancer or a noncancerous condition. Tumor markers may be used for diagnosis, treatment planning, and/or treatment monitoring. For more information, read the Tumor Markers for Breast Cancer. Additional tests The doctor may order additional tests (depending on the individual’s medical history and results of the physical examination) to evaluate the stage of the cancer. Read the Staging section for more information. These tests are not recommended for all patients.
To learn more about what to expect during common diagnostic tests, read Cancer.Net: Tests and Procedures. To learn about the terms used in this section, read the Cancer.Net Feature: Cancer Terms to Know: Newly Diagnosed. Staging
Staging is a way of describing a cancer, such as where it is located, if or where it has spread, and if it is affecting the functions of other organs in the body. Doctors use diagnostic tests to determine the cancer's stage, so staging may not be complete until all the tests are finished. Knowing the stage helps the doctor to decide what kind of treatment is best and can help predict a patient's prognosis. There are different stage descriptions for different types of cancer. One tool that doctors use to describe the stage is the TNM system. This system uses three criteria to judge the stage of the cancer: the tumor itself, the lymph nodes around the tumor, and if the tumor has spread to other parts of the body. The results are combined to determine the stage of cancer for each person. There are five stages: stage 0 (zero) and stages I through IV (one through four). The stage provides a common way of describing the cancer so doctors can work together to plan the best treatments. TNM is an abbreviation for tumor (T), node (N), and metastasis (M). Doctors look at these three factors to determine the stage of cancer:
Inflammatory breast cancer is generally considered stage IIIb breast cancer at a minimum at the time of diagnosis. For more complete breast cancer staging information, see the Staging section of the Cancer.Net Guide to Breast Cancer. Stage IIIb: The cancer has spread to the chest wall or caused swelling or ulceration of the breast. It may or may not have spread to the lymph nodes under the arm, but it has not spread to other parts of the body (T4, N0, N1, N2, M0). Stage IV: The cancer can be any size and has spread to distant sites in the body, usually the bones, lung, liver, or brain (any T, any N, M1). Recurrent breast cancer Recurrent cancer is cancer that comes back after treatment. Inflammatory breast cancer may come back in the breast (called a local recurrence), in the chest wall, or in another part of the body (called a distant metastasis), including distant organs (such as the lungs or liver), bones, or other lymph nodes. Used with permission of the American Joint Committee on Cancer (AJCC), Chicago, Illinois. The original source for this material is the AJCC Cancer Staging Manual, Sixth Edition (2002) published by Springer-Verlag New York, www.springer-ny.com. Treatment
The treatment of inflammatory breast cancer depends on the size and location of the tumor, whether the cancer has spread, and the woman’s overall health. In many cases, a team of doctors will work with the patient to determine the best treatment plan. For inflammatory breast cancer, treatment often includes surgery, radiation therapy, chemotherapy, and hormone therapy. Each treatment option is described below. Inflammatory breast cancer is typically considered a late-stage breast cancer and, therefore, treated aggressively. This section outlines treatments that are the standard of care (the best treatments available) for this specific type of cancer. Patients are also encouraged to consider clinical trials when making treatment plan decisions. A clinical trial is a research study to test a new treatment to prove it is safe, effective, and possibly better than standard treatment. Your doctor can help you review all treatment options. For more information, read the Clinical trials section. Chemotherapy Chemotherapy is the use of drugs to kill cancer cells. Systemic chemotherapy is delivered through the bloodstream, targeting cancer cells throughout the body. The side effects of chemotherapy depend on the individual and the drug and the dose used, but can include fatigue, hair loss, risk of infection, nausea and vomiting, loss of appetite, and diarrhea. These side effects usually go away once treatment is finished. Rarely, long-term side effects may occur, such as heart damage, nerve damage, or secondary cancers, but studies have shown that these side effects do not shorten a woman’s survival time. Chemotherapy may be given orally (by mouth) or intravenously (injected into a vein) and is usually given in cycles. Chemotherapy generally does not require a hospital stay; it is given in an outpatient setting. Chemotherapy may be neoadjuvant therapy (given before surgery to shrink a large tumor) or adjuvant therapy (given after surgery to reduce the risk that the cancer returns). Chemotherapy may also be given at the time of a breast cancer recurrence. Patients in clinical trials may be offered new drugs or new combinations of existing drugs. Chemotherapy may be the first treatment given for inflammatory breast cancer, especially because cancer cells may have already spread to other parts of the body. Chemotherapy can reduce the size of the tumor and the swelling in the breast, increasing the likelihood of successful surgery. The medications used to treat cancer are continually being evaluated. Talking with your doctor is often the best way to learn about the medications prescribed for you, their purpose, and their potential side effects or interactions. Learn more about your prescriptions through Cancer.Net’s Drug Information Resources , which provides links to multiple drug databases. Surgery The type of surgery for inflammatory breast cancer depends on the stage of the cancer. Because inflammatory breast cancer grows quickly, a mastectomy (removal of the entire breast), is often done. In some cases, lumpectomy (removal of the tumor and a disease-free area [margin] of tissue around it), is possible. For more information about considerations after breast cancer surgery, read after a mastectomy and preventing lymphedema after breast cancer treatment, breast reconstruction, and choosing a breast prosthesis. Radiation therapy Radiation therapy is the use of high-energy x-rays or other particles to kill cancer cells. Adjuvant radiation therapy is given regularly for a number of weeks after a lumpectomy or partial mastectomy to eliminate any remaining cancer cells near the tumor site or elsewhere within the breast. Adjuvant radiation therapy is also recommended for some women after a mastectomy depending upon the size of their tumor, number of cancerous lymph nodes under the arm, and width of the tissue margin around the tumor removed by the surgeon. Adjuvant radiation therapy is effective in reducing the chance of breast cancer returning in both the breast and the chest wall. Neoadjuvant radiation therapy is radiation therapy given before surgery to shrink a large tumor, which makes it easier to remove, although this approach is rare. Radiation therapy can cause side effects, including fatigue, swelling, and skin changes. A small amount of the lung can be affected by the radiation, although the risk of pneumonitis, or a radiation-related pneumonia, is rare. In the past, with older equipment and techniques of radiation therapy, women treated for left-sided breast cancers had a small increase in the long-term risk of heart disease. Modern techniques are now able to spare most of the heart from radiation damage. Although exposure to radiation is thought to be a risk factor for cancer after many years, less than one in 500 survivors will develop a different kind of cancer, other than a breast cancer, within the area that was treated. Clinical trials comparing lumpectomy and adjuvant radiation therapy with mastectomy have not shown a difference in the number of patients developing or dying of other cancers within a 20-year time span. The most common type of radiation treatment is called external beam radiation therapy, which is radiation therapy given from a machine outside the body. When radiation treatment is given using implants, it is called internal radiation therapy or brachytherapy. In this treatment, small radioactive pellets are placed in or near the site of the breast tumor within plastic catheters placed temporarily in the breast. A balloon catheter placed near the breast that delivers radiation therapy (called Mammosite) is another type of radiation therapy. For more information on radiation therapy, read the Cancer.Net Feature: Understanding Radiation Therapy, the Cancer.Net Feature: Radiation Therapy—Your Personal Experience, and the Cancer.Net Feature: Side Effects of Radiation Therapy. Hormone therapy Hormone therapy is useful to manage a tumor that tests positive for either estrogen or progesterone receptors for both early-stage and metastatic cancer. This type of tumor uses hormones to fuel its growth. Blocking the hormones usually limits the growth of the tumor. If it is determined that the tumor is hormone receptor-positive (uses estrogen or progesterone to grow [see Diagnosis]), then adjuvant hormone treatment may be used alone or after chemotherapy. Examples of hormone therapy used as adjuvant therapy are tamoxifen (Nolvadex), anastrozole (Arimidex), letrozole (Femara), and exemestane (Aromasin). Tamoxifen is the drug that researchers have studied the longest for use as a hormone therapy. It blocks estrogen from binding to breast cancer cells. It has been shown to be effective for reducing the risk of recurrence in the treated breast, the risk of developing cancer in the other breast, and the risk of developing cancer in women with no history of the disease but who are at higher than average risk for breast cancer. Current research shows that there is no benefit of taking tamoxifen longer than five years. The side effects of tamoxifen include hot flashes, a small increased risk of uterine (endometrial) cancer and uterine sarcoma, and an increase in the risk of blood clots. Tamoxifen can be effective for both premenopausal and postmenopausal women. In postmenopausal women who have an increased risk of developing breast cancer, raloxifene has shown to be another hormone therapy that is as effective as tamoxifen in preventing invasive breast cancer, but not as effective in preventing noninvasive cancer, such as ductal carcinoma in situ (DCIS). The side effects of raloxifene include a small risk of blood clots, leg and joint pain, hot flashes, pain during sexual intercourse, and vaginal dryness. Raloxifene has not been studied in premenopausal women, and it is not considered a substitute for tamoxifen for adjuvant therapy for women with hormone receptor-positive breast cancer. An aromatase inhibitor (AI) decreases the amount of estrogen in postmenopausal women by blocking the aromatase enzyme, which is needed to make estrogen. These drugs include anastrozole, letrozole, and exemestane. The side effects of AIs may include joint pain and an increased risk of fractures (broken bones). Clinical trials are evaluating whether women benefit from an AI after tamoxifen, or by taking an AI for more than five years. For more information about AIs, read the ASCO Technology Assessment for Patients: Aromatase Inhibitors for Early Breast Cancer. Recurrent and metastatic breast cancer Breast cancer is called recurrent if the cancer has come back after it was first diagnosed and treated. It may come back in the breast (a local recurrence); in the chest wall; or in another part of the body, including distant organs (such as the lungs, liver, and bones). Some patients live years after a recurrence of breast cancer. Breast cancer may also spread to other organs such as the brain, the opposite breast, adrenal glands, spleen, and ovaries and is called metastatic breast cancer. This type of cancer is treatable, but not curable. The goal of treatment for advanced disease is to achieve remission (temporary or permanent absence of disease) or slow the growth of the tumor. Generally, a recurrence is detected when a person has symptoms. Even though there are tests that may detect a metastatic recurrence before the onset of symptoms, research shows that having such tests does not improve the response to treatments used for advanced disease, nor do they prolong life. Signs and symptoms depend on the site of the recurrence and may include:
Other symptoms may be related to the location of metastasis and may include changes in vision, changes in energy levels, feeling ill, or extreme fatigue. A biopsy of the recurrent site is often recommended to be certain of the diagnosis and to check for ER, PR, and HER2 status, because this may have changed from the time of the original diagnosis. The treatment of metastatic or recurrent breast cancer depends on the previous treatment(s) and the characteristics of the tumor (such as ER, PR, and HER2 status). Once metastatic disease is detected, the treatment may involve surgery to remove the metastasis and/or chemotherapy, hormone therapy, targeted therapy, and radiation therapy (if it hasn’t been already given) to control it. In some circumstances, radiation therapy may also be given to relieve symptoms. Treatment guides from ASCO for breast cancer include Aromatase Inhibitors for Early Breast Cancer,Bisphosphonates for Breast Cancer, Follow-Up Care for Breast Cancer, HER2 Testing for Breast Cancer, Sentinel Lymph Node Biopsy for Early-Stage Breast Cancer, and Tumor Markers for Breast Cancer. Clinical Trials Resources
Doctors and scientists are always looking for better ways to treat patients with inflammatory breast cancer. A clinical trial is a way to test a new treatment to prove that it is safe, effective, and possibly better than a standard treatment. Patients who participate in clinical trials are among the first to receive new treatments, such as new chemotherapy, before they are widely available. However, there is no guarantee that the new treatment will be safe, effective, or better than a standard treatment. Patients decide to participate in clinical trials for many reasons. For some patients, a clinical trial is the best treatment option available. Because standard treatments are not perfect, patients are often willing to face the added uncertainty of a clinical trial in the hope of a better result. Other patients volunteer for clinical trials because they know that this is the only way to make progress in treating breast cancer, such as finding new drugs. Even if they do not benefit directly from the clinical trial, their participation may benefit future patients with inflammatory breast cancer. To join a clinical trial, patients must complete a learning process known as informed consent. During informed consent, the doctor should list all of the patient's options, so that the person understands the standard treatment, and how the new treatment differs from the standard treatment. The doctor must also list all of the risks of the new treatment, which may or may not be different from the risks of standard treatment. Finally, the doctor must explain what will be required of each patient in order to participate in the clinical trial, including the number of doctor visits, tests, and the schedule of treatment. Learn more about clinical trials, including patient safety, phases of a clinical trial, deciding to participate in a clinical trial, questions to ask the research team, and links to find cancer clinical trials. Side Effects of Cancer and Cancer Treatment
Cancer and its treatment can cause a variety of side effects. However, doctors have made major strides in recent years in reducing pain, nausea and vomiting, infection, fatigue, and other physical side effects of cancer treatments. Many treatments used today are less intensive but as effective as treatments used in the past. Doctors also have many ways to provide relief to patients when such side effects do occur. Fear of treatment side effects is common after a diagnosis of cancer, but it may be helpful to know that preventing and controlling side effects is a major focus of your health-care team. Before treatment begins, talk with your doctor about possible side effects of the specific treatments you will be receiving. The specific side effects that can occur depend on a variety of factors, including the type of cancer, its location, the individual treatment plan (including the length and dosage of treatment), and the person’s overall health. Ask your doctor which side effects are most likely to happen (and which are not), when side effects are likely to occur, and how they will be addressed by the health-care team if they do happen. Also, be sure to communicate with the doctor about side effects you experience during and after treatment. For more information on the most common side effects of cancer and different treatments, along with ways to prevent or control them, visit Cancer.Net’s section on Managing Side Effects , based on ASCO’s curriculum. In addition to physical side effects, there may be psychosocial (emotional and social) effects as well. Learn more about the importance of addressing these needs in Cancer.Net’s section on Caring for the Whole Patient. For more information on late effects or long-term side effects, please read the After Treatment section or talk with your doctor. After Treatment
After treatment for breast cancer ends, talk with your doctor about developing a follow-up care plan. This plan may include regular physical examinations and/or medical tests to monitor your recovery for the coming months and years. The recommendations for breast cancer follow-up care usually include regular physical examinations and mammograms. Specific information can be found in the Follow-Up Care for Breast Cancer. In addition, ASCO offers forms to help keep track of the breast cancer treatment you received and develop a survivorship care plan once treatment ends. Read more about the ASCO Cancer Treatment Summaries. Breast cancer can come back in the breast or other areas of the body. The symptoms of a cancer recurrence include a new lump in the breast, under the arm, or along the chest wall; bone pain or fractures; headaches or seizures; chronic coughing or trouble breathing; extreme fatigue; and/or feeling ill. Talk with your doctor if you have these or other symptoms. The possibility of recurrence is a common concern among cancer survivors; learn more about Coping With Fear of Recurrence. After surgery (mastectomy or lumpectomy) to treat breast cancer, the breast may be scarred and may have a different shape or size than before surgery. If lymph nodes were removed as part of the surgery or affected during treatment, lymphedema (swelling of the hand and/or arm) may occur. Read more about preventing lymphedema after breast cancer treatment, after a mastectomy, breast reconstruction, and choosing a breast prosthesis. Some patients experience breathlessness, a dry cough, and/or chest pain two to three months after finishing radiation therapy because the treatment can cause swelling and fibrosis (hardening or thickening) of the lungs. These symptoms are usually temporary. Talk with your doctor if you develop any new symptoms after radiation therapy or if the side effects are not going away. Women taking tamoxifen should have yearly pelvic exams, because this drug can increase the risk of uterine cancer. Tell your doctor or nurse if you notice any abnormal vaginal bleeding or other new symptoms. Women who are taking an aromatase inhibitor, such as anastrozole, exemestane, or letrozole, should have a bone density test before they start treatment and as recommended by their doctor, as these drugs may cause some bone weakness or bone loss. Women recovering from breast cancer are encouraged to follow established guidelines for good health, such as maintaining a healthy weight, not smoking, eating a balanced diet, and having recommended cancer screening tests. Talk with your doctor to develop a plan that is best for your needs. Moderate physical activity can help rebuild your strength and energy level and lowers the risk of recurrence. Your doctor can help you create a safe exercise plan based upon your needs, physical abilities, and fitness level. Learn more about healthy living after cancer. Find out more about common terms used after cancer treatment is complete. Current Research
Research for inflammatory breast cancer is ongoing. The following advances may still be under investigation in clinical trials and may not be approved or available at this time. Always discuss all diagnostic and treatment options with your doctor. The latest trend in research is looking at the effectiveness of antibodies combined with chemotherapy for people with inflammatory breast cancer. Trastuzumab combined with chemotherapy is promising for treatment before surgery of people with inflammatory breast cancer with a cancer that has extra copies of HER2. Also, the drug bevacizumab (Avastin), used in combination with the established drugs doxorubicin (Adriamycin, Rubex) and docetaxel (Taxotere), is being evaluated. Bevacizumab is a new drug that works by blocking the growth of new blood vessels that can bring nutrients to the cancer cells, called anti-angiogenesis. Questions to Ask the Doctor
Regular communication with your doctor is important in making informed decisions about your health care. Consider asking the following questions of your doctor:
For additional questions, see the Cancer.Net Guide to Breast Cancer section. Patient Information Resources
Inflammatory Breast Cancer Research Foundation National Organization for Rare Disorders Rare Cancer Alliance American Society of Breast Disease Breastcancer.org Breast Cancer Network of Strength Breastlink Fertile Hope FORCE: Facing Our Risk of Cancer Empowered HER2 Support Group Living Beyond Breast Cancer Mothers Supporting Daughters with Breast Cancer National Breast Cancer Coalition National Cancer Institute National Comprehensive Cancer Network National Lymphedema Network Nueva Vida, Inc. SHARE: Self-help for Women with Breast or Ovarian Cancer Sisters Network, Inc. Susan G. Komen for the Cure Young Survival Coalition View all of Cancer.Net's Patient Information Resources. |
