Key Points Inflammatory breast cancer (IBC) is a rare but very aggressive type of breast cancer (see Question 1). IBC usually grows rapidly and often spreads to other parts of the body; symptoms include redness, swelling, and warmth in the breast (see Questions 2 and 3). Treatment for IBC usually starts with chemotherapy, which is generally followed by surgery, radiation, targeted therapy, and/or hormone therapy (see Question 4). People with IBC are encouraged to enroll in clinical trials (research studies with people) that explore new treatments (see Question 5).

1. What is inflammatory breast cancer (IBC)?

Inflammatory breast cancer is a rare but very aggressive type of breast cancer in which the cancer cells block the lymph vessels in the skin of the breast. This type of breast cancer is called “inflammatory” because the breast often looks swollen and red, or “inflamed.” IBC accounts for 1 to 5 percent of all breast cancer cases in the United States (1). It tends to be diagnosed in younger women compared to non-IBC breast cancer. It occurs more frequently and at a younger age in African Americans than in Whites. Like other types of breast cancer, IBC can occur in men, but usually at an older age than in women. Some studies have shown an association between family history of breast cancer and IBC, but more studies are needed to draw firm conclusions (2).

2. What are the symptoms of IBC?

Symptoms of IBC may include redness, swelling, and warmth in the breast, often without a distinct lump in the breast. The redness and warmth are caused by cancer cells blocking the lymph vessels in the skin. The skin of the breast may also appear pink, reddish purple, or bruised. The skin may also have ridges or appear pitted, like the skin of an orange (called peau d'orange), which is caused by a buildup of fluid and edema (swelling) in the breast. Other symptoms include heaviness, burning, aching, increase in breast size, tenderness, or a nipple that is inverted (facing inward) (3). These symptoms usually develop quickly—over a period of weeks or months. Swollen lymph nodes may also be present under the arm, above the collarbone, or in both places. However, it is important to note that these symptoms may also be signs of other conditions such as infection, injury, or other types of cancer (1).

3. How is IBC diagnosed?

Diagnosis of IBC is based primarily on the results of a doctor's clinical examination (1). Biopsy, mammogram, and breast ultrasound are used to confirm the diagnosis. IBC is classified as either stage IIIB or stage IV breast cancer (2). Stage IIIB breast cancers are locally advanced; stage IV breast cancer is cancer that has spread to other organs. IBC tends to grow rapidly, and the physical appearance of the breast of patients with IBC is different from that of patients with other stage III breast cancers. IBC is an especially aggressive, locally advanced breast cancer.

Cancer staging describes the extent or severity of an individual's cancer. (More information on staging is available in the National Cancer Institute (NCI) fact sheet Staging: Questions and Answers at http://www.cancer.gov/cancertopics/factsheet/Detection/staging on the Internet.) Knowing a cancer's stage helps the doctor develop a treatment plan and estimate prognosis (the likely outcome or course of the disease; the chance of recovery or recurrence).

4. How is IBC treated?

Treatment consisting of chemotherapy, targeted therapy, surgery, radiation therapy, and hormonal therapy is used to treat IBC. Patients may also receive supportive care to help manage the side effects of the cancer and its treatment. Chemotherapy (anticancer drugs) is generally the first treatment for patients with IBC, and is called neoadjuvant therapy. Chemotherapy is systemic treatment, which means that it affects cells throughout the body. The purpose of chemotherapy is to control or kill cancer cells, including those that may have spread to other parts of the body.

After chemotherapy, patients with IBC may undergo surgery and radiation therapy to the chest wall. Both radiation and surgery are local treatments that affect only cells in the tumor and the immediately surrounding area. The purpose of surgery is to remove the tumor from the body, while the purpose of radiation therapy is to destroy remaining cancer cells. Surgery to remove the breast (or as much of the breast tissue as possible) is called a mastectomy. Lymph node dissection (removal of the lymph nodes in the underarm area for examination under a microscope) is also done during this surgery.

After initial systemic and local treatment, patients with IBC may receive additional systemic treatments to reduce the risk of recurrence (cancer coming back). Such treatments may include additional chemotherapy, hormonal therapy (treatment that interferes with the effects of the female hormone estrogen, which can promote the growth of breast cancer cells), targeted therapy (such as trastuzumab, also known as Herceptin®), or all three. Trastuzumab is administered to patients whose tumors overexpress the HER–2 tumor protein. More information about Herceptin and the HER–2 protein is available in the NCI fact sheet Herceptin® (Trastuzumab): Questions and Answers, which can be found at http://www.cancer.gov/cancertopics/factsheet/therapy/herceptin on the Internet.

Supportive care is treatment given to improve the quality of life of patients who have a serious or life-threatening disease, such as cancer. It prevents or treats as early as possible the symptoms of the disease, side effects caused by treatment of the disease, and psychological, social, and spiritual problems related to the disease or its treatment. For example, compression garments may be used to treat lymphedema (swelling caused by excess fluid buildup) resulting from radiation therapy or the removal of lymph nodes. Additionally, meeting with a social worker, counselor, or member of the clergy can be helpful to those who want to talk about their feelings or discuss their concerns. A social worker can often suggest resources for help with recovery, emotional support, financial aid, transportation, or home care.

5. Are clinical trials (research studies with people) available? Where can people get more information about clinical trials?

Yes. The NCI is sponsoring clinical trials that are designed to find new treatments and better ways to use current treatments. Before any new treatment can be recommended for general use, doctors conduct clinical trials to find out whether the treatment is safe for patients and effective against the disease. Participation in clinical trials is a treatment option for many patients with IBC, and all patients with IBC are encouraged to consider treatment in a clinical trial.

People interested in taking part in a clinical trial should talk with their doctor. Information about clinical trials is available from the NCI's Cancer Information Service (CIS) (see below) at 1–800–4–CANCER and in the NCI booklet Taking Part in Cancer Treatment Research Studies, which is available at http://www.cancer.gov/publications on the Internet . This booklet describes how research studies are carried out and explains their possible benefits and risks. Further information about clinical trials is available at http://www.cancer.gov/clinicaltrials on the NCI's Web site. The Web site offers detailed information about specific ongoing studies by linking to PDQ®, the NCI's comprehensive cancer information database. The CIS also provides information from PDQ.

6. What is the prognosis for patients with IBC?

Prognosis describes the likely course and outcome of a disease—that is, the chance that a patient will recover or have a recurrence. IBC is more likely to have metastasized (spread to other areas of the body) at the time of diagnosis than non-IBC cases (3). As a result, the 5-year survival rate for patients with IBC is between 25 and 50 percent, which is significantly lower than the survival rate for patients with non-IBC breast cancer. It is important to keep in mind, however, that these statistics are averages based on large numbers of patients. Statistics cannot be used to predict what will happen to a particular patient because each person's situation is unique. Patients are encouraged to talk to their doctors about their prognosis given their particular situation.

7. Where can a person find more information about breast cancer and its treatment?

To learn more about IBC, other types of breast cancer, and breast health in general, please refer to the following resources:

• NCI's Breast Cancer Home Page (http://www.cancer.gov/breast/)
• Breast Cancer (PDQ®): Treatment (http://www.cancer.gov/cancertopics/pdq/treatment/breast/patient/)
• Understanding Breast Changes: A Health Guide for All Women (http://www.cancer.gov/cancertopics/understanding-breast-changes)
• What You Need To Know About™ Breast Cancer (http://www.cancer.gov/cancertopics/wyntk/breast)

Selected References

1. Merajver SD, Sabel MS. Inflammatory breast cancer. In: Harris JR, Lippman ME, Morrow M, Osborne CK, editors. Diseases of the Breast. 3^rd ed. Philadelphia: Lippincott Williams and Wilkins, 2004.

2. Anderson W, Schairer C, Chen B, Hance K, Levine P. Epidemiology of inflammatory breast cancer (IBC). Breast Disease 2005; 22:9–23.
3. Chittoor SR, Swain SM. Locally advanced breast cancer: Role of medical oncology. In: Bland KI, Copeland EM, editors. The Breast: Comprehensive Management of Benign and Malignant Diseases. Vol. 2. 2^nd ed. Philadelphia: W.B. Saunders Company, 1998.

# # #

Related NCI materials and Web pages:
• National Cancer Institute Fact Sheet 2.1, Cancer Information Sources ¹
  (http://www.cancer.gov/cancertopics/factsheet/Information/sources)
• National Cancer Institute Fact Sheet 5.32, Staging: Questions and Answers ²
  (http://www.cancer.gov/cancertopics/factsheet/Detection/staging)
• National Cancer Institute Fact Sheet 7.1, Radiation Therapy for Cancer: Questions and Answers ³
  (http://www.cancer.gov/cancertopics/factsheet/Therapy/radiation)
• National Cancer Institute Fact Sheet 7.2, Biological Therapies for Cancer: Questions and Answers ⁴
  (http://www.cancer.gov/cancertopics/factsheet/Therapy/biological)
• National Cancer Institute Fact Sheet 7.45, Herceptin® (Trastuzumab): Questions and Answers ⁵
  (http://www.cancer.gov/cancertopics/factsheet/therapy/herceptin)
• Chemotherapy and You: Support for People With Cancer ⁶ (http://www.cancer.gov/cancertopics/chemotherapy-and-you)
• Taking Part in Cancer Treatment Research Studies ⁷ (http://www.cancer.gov/clinicaltrials/Taking-Part-in-Cancer-Treatment-Research-Studies)

For more help, contact:

NCI's Cancer Information Service
Telephone (toll-free): 1–800–4–CANCER (1–800–422–6237)
TTY (toll-free): 1–800–332–8615
LiveHelp^® online chat: https://cissecure.nci.nih.gov/livehelp/welcome.asp

Glossary Terms

A quickly growing cancer.

The removal of cells or tissues for examination by a pathologist. The pathologist may study the tissue under a microscope or perform other tests on the cells or tissue. There are many different types of biopsy procedures. The most common types include: (1) incisional biopsy, in which only a sample of tissue is removed; (2) excisional biopsy, in which an entire lump or suspicious area is removed; and (3) needle biopsy, in which a sample of tissue or fluid is removed with a needle. When a wide needle is used, the procedure is called a core biopsy. When a thin needle is used, the procedure is called a fine-needle aspiration biopsy.

Glandular organ located on the chest. The breast is made up of connective tissue, fat, and breast tissue that contains the glands that can make milk. Also called mammary gland.

The Cancer Information Service is the National Cancer Institute's link to the public, interpreting and explaining research findings in a clear and understandable manner, and providing personalized responses to specific questions about cancer. Access the CIS by calling 1-800-4-CANCER (1-800-422-6237), or by using the LiveHelp instant-messaging service at https://cissecure.nci.nih.gov/livehelp/welcome.asp. Also called CIS.

The individual unit that makes up the tissues of the body. All living things are made up of one or more cells.

Treatment with drugs that kill cancer cells.

The muscles, bones, and joints that make up the area of the body between the neck and the abdomen.

Ordained individuals who perform spiritual and/or religious functions.

Having to do with the examination and treatment of patients.

A type of research study that tests how well new medical approaches work in people. These studies test new methods of screening, prevention, diagnosis, or treatment of a disease. Also called clinical study.

The process of identifying a disease, such as cancer, from its signs and symptoms.

Any substance, other than food, that is used to prevent, diagnose, treat or relieve symptoms of a disease or abnormal condition. Also refers to a substance that alters mood or body function, or that can be habit-forming or addictive, especially a narcotic.

Swelling caused by excess fluid in body tissues.

A type of hormone made by the body that helps develop and maintain female sex characteristics and the growth of long bones. Estrogens can also be made in the laboratory. They may be used as a type of birth control and to treat symptoms of menopause, menstrual disorders, osteoporosis, and other conditions.

A record of a person's current and past illnesses, and those of his or her parents, brothers, sisters, children, and other family members. A family history shows the pattern of certain diseases in a family, and helps to determine risk factors for those and other diseases.

Liquid.

A monoclonal antibody that binds to HER2 (human epidermal growth factor receptor 2), and can kill HER2-positive cancer cells. Monoclonal antibodies are made in the laboratory and can locate and bind to substances in the body, including cancer cells. Herceptin is used to treat breast cancer that is HER2-positive and has spread after treatment with other drugs. It is also used with other anticancer drugs to treat HER2-positive breast cancer after surgery. Herceptin is also being studied in the treatment of other types of cancer. Also called trastuzumab.

Treatment that adds, blocks, or removes hormones. For certain conditions (such as diabetes or menopause), hormones are given to adjust low hormone levels. To slow or stop the growth of certain cancers (such as prostate and breast cancer), synthetic hormones or other drugs may be given to block the body’s natural hormones. Sometimes surgery is needed to remove the gland that makes a certain hormone. Also called endocrine therapy, hormone therapy, and hormone treatment.

Treatment that adds, blocks, or removes hormones. For certain conditions (such as diabetes or menopause), hormones are given to adjust low hormone levels. To slow or stop the growth of certain cancers (such as prostate and breast cancer), synthetic hormones or other drugs may be given to block the body’s natural hormones. Sometimes surgery is needed to remove the gland that makes a certain hormone. Also called endocrine therapy, hormonal therapy, and hormone treatment.

Invasion and multiplication of germs in the body. Infections can occur in any part of the body and can spread throughout the body. The germs may be bacteria, viruses, yeast, or fungi. They can cause a fever and other problems, depending on where the infection occurs. When the body’s natural defense system is strong, it can often fight the germs and prevent infection. Some cancer treatments can weaken the natural defense system.

A type of breast cancer in which the breast looks red and swollen and feels warm. The skin of the breast may also show the pitted appearance called peau d'orange (like the skin of an orange). The redness and warmth occur because the cancer cells block the lymph vessels in the skin.

Cancer that has spread from where it started to nearby tissue or lymph nodes.

A rounded mass of lymphatic tissue that is surrounded by a capsule of connective tissue. Lymph nodes filter lymph (lymphatic fluid), and they store lymphocytes (white blood cells). They are located along lymphatic vessels. Also called lymph gland.

A surgical procedure in which the lymph nodes are removed and examined to see whether they contain cancer. For a regional lymph node dissection, some of the lymph nodes in the tumor area are removed; for a radical lymph node dissection, most or all of the lymph nodes in the tumor area are removed. Also called lymphadenectomy.

A thin tube that carries lymph (lymphatic fluid) and white blood cells through the lymphatic system. Also called lymphatic vessel.

A condition in which excess fluid collects in tissue and causes swelling. It may occur in the arm or leg after lymph vessels or lymph nodes in the underarm or groin are removed or treated with radiation.

An x-ray of the breast.

Surgery to remove the breast (or as much of the breast tissue as possible).

To spread from one part of the body to another. When cancer cells metastasize and form secondary tumors, the cells in the metastatic tumor are like those in the original (primary) tumor.

The National Cancer Institute, part of the National Institutes of Health of the United States Department of Health and Human Services, is the Federal Government's principal agency for cancer research. The National Cancer Institute conducts, coordinates, and funds cancer research, training, health information dissemination, and other programs with respect to the cause, diagnosis, prevention, and treatment of cancer. Access the National Cancer Institute Web site at http://www.cancer.gov. Also called NCI.

Treatment given as a first step to shrink a tumor before the main treatment, which is usually surgery, is given. Examples of neoadjuvant therapy include chemotherapy, radiation therapy, and hormone therapy.

In anatomy, the small raised area in the center of the breast through which milk can flow to the outside.

A part of the body that performs a specific function. For example, the heart is an organ.

In biology, to make too many copies of a protein or other substance. Overexpression of certain proteins or other substances may play a role in cancer development.

PDQ is an online database developed and maintained by the National Cancer Institute. Designed to make the most current, credible, and accurate cancer information available to health professionals and the public, PDQ contains peer-reviewed summaries on cancer treatment, screening, prevention, genetics, complementary and alternative medicine, and supportive care; a registry of cancer clinical trials from around the world; and directories of physicians, professionals who provide genetics services, and organizations that provide cancer care. Most of this information, and more specific information about PDQ, can be found on the NCI's Web site at http://www.cancer.gov/cancertopics/pdq. Also called Physician Data Query.

The likely outcome or course of a disease; the chance of recovery or recurrence.

A molecule made up of amino acids that are needed for the body to function properly. Proteins are the basis of body structures such as skin and hair and of substances such as enzymes, cytokines, and antibodies.

The overall enjoyment of life. Many clinical trials assess the effects of cancer and its treatment on the quality of life. These studies measure aspects of an individual’s sense of well-being and ability to carry out various activities.

Energy released in the form of particle or electromagnetic waves. Common sources of radiation include radon gas, cosmic rays from outer space, medical x-rays, and energy given off by a radioisotope (unstable form of a chemical element that releases radiation as it breaks down and becomes more stable).

The use of high-energy radiation from x-rays, gamma rays, neutrons, protons, and other sources to kill cancer cells and shrink tumors. Radiation may come from a machine outside the body (external-beam radiation therapy), or it may come from radioactive material placed in the body near cancer cells (internal radiation therapy). Systemic radiation therapy uses a radioactive substance, such as a radiolabeled monoclonal antibody, that travels in the blood to tissues throughout the body. Also called irradiation and radiotherapy.

Cancer that has recurred (come back), usually after a period of time during which the cancer could not be detected. The cancer may come back to the same place as the original (primary) tumor or to another place in the body. Also called recurrent cancer.

A problem that occurs when treatment affects healthy tissues or organs. Some common side effects of cancer treatment are fatigue, pain, nausea, vomiting, decreased blood cell counts, hair loss, and mouth sores.

A professional trained to talk with people and their families about emotional or physical needs, and to find them support services.

Having to do with deep, often religious, feelings and beliefs, including a person’s sense of peace, purpose, connection to others, and beliefs about the meaning of life.

The extent of a cancer in the body. Staging is usually based on the size of the tumor, whether lymph nodes contain cancer, and whether the cancer has spread from the original site to other parts of the body.

Stage III breast cancer is divided into stages IIIA, IIIB, and IIIC. In stage IIIB, the tumor may be any size and cancer (1) has spread to the chest wall and/or the skin of the breast; and (2) may have spread to axillary lymph nodes that may be attached to each other or to other structures or the cancer may have spread to lymph nodes near the breastbone.

Cancer has spread to other organs of the body, most often the bones, lungs, liver, or brain.

Performing exams and tests to learn the extent of the cancer within the body, especially whether the disease has spread from the original site to other parts of the body. It is important to know the stage of the disease in order to plan the best treatment.

Care given to improve the quality of life of patients who have a serious or life-threatening disease. The goal of supportive care is to prevent or treat as early as possible the symptoms of a disease, side effects caused by treatment of a disease, and psychological, social, and spiritual problems related to a disease or its treatment. Also called comfort care, palliative care, and symptom management.

A procedure to remove or repair a part of the body or to find out whether disease is present. An operation.

The percentage of people in a study or treatment group who are alive for a certain period of time after they were diagnosed with or treated for a disease, such as cancer. The survival rate is often stated as a five-year survival rate, which is the percentage of people in a study or treatment group who are alive five years after diagnosis or treatment. Also called overall survival rate.

An indication that a person has a condition or disease. Some examples of symptoms are headache, fever, fatigue, nausea, vomiting, and pain.

Treatment using substances that travel through the bloodstream, reaching and affecting cells all over the body.

A type of treatment that uses drugs or other substances, such as monoclonal antibodies, to identify and attack specific cancer cells without harming normal cells. Targeted therapy may be less harmful to normal cells than other types of cancer treatments

A group or layer of cells that work together to perform a specific function.

A monoclonal antibody that binds to HER2 (human epidermal growth factor receptor 2), and can kill HER2-positive cancer cells. Monoclonal antibodies are made in the laboratory and can locate and bind to substances in the body, including cancer cells. Trastuzumab is used to treat breast cancer that is HER2-positive and has spread after treatment with other drugs. It is also used with other anticancer drugs to treat HER2-positive breast cancer after surgery. Trastuzumab is also being studied in the treatment of other types of cancer. Also called Herceptin.

An abnormal mass of tissue that results when cells divide more than they should or do not die when they should. Tumors may be benign (not cancerous), or malignant (cancerous). Also called neoplasm.

A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echo patterns are shown on the screen of an ultrasound machine, forming a picture of body tissues called a sonogram. Also called ultrasonography.