Inflammatory breast cancer (IBC) is a rare type of breast cancer. IBC leads to unusual symptoms and is more difficult to diagnose than many other types of breast cancer. The condition’s symptoms can include discoloration, swelling, tenderness, and dimpling of the skin. It can be misdiagnosed as mastitis, a skin infection on the breast. IBC grows and spreads fast and needs to be treated quickly.

About 1 percent to 5 percent of people with breast cancer have IBC. According to the American Cancer Society, Black women are more likely to be diagnosed with IBC than white women in the United States. Additionally, people who develop this cancer tend to be younger. Across all types of breast cancer, the average age at diagnosis in the U.S. is 63 years old. However, the average age of IBC diagnosis in the U.S. is 57 years old for white women and 52 for Black women, according to Breastcancer.org.

What Causes IBC?

Any cell in the body can undergo gene changes that make it turn cancerous. These changes can make a cell grow out of control. Inflammatory breast cancer develops when gene changes occur in a cell located in the lobule (a gland that makes breast milk) or near a duct (a tube that transports the milk).

As the original cancer cell grows and divides, it creates many more cancer cells that block lymph vessels. Lymph vessels are a part of the lymphatic system — the collection of organs and tissues that fights infection, removes waste, and controls levels of fluid around the body. When breast cancer cells block the lymph vessels, lymph fluid can’t properly drain out of the breast. As lymph fluid builds up, the breast swells and changes in appearance. These cells can also spread to the skin, causing an inflamed appearance.

Inflammatory Breast Cancer Risk Factors

Certain factors can increase a person’s chances of being diagnosed with IBC. These include:

• Having a family history of breast cancer
• Having a personal history of another type of breast cancer
• Having dense breast tissue (having more ducts and supportive tissue and less fatty tissue)
• Starting menstruation before the age of 12
• Never having been pregnant
• Having a first pregnancy after the age of 30
• Being obese
• Having gone through radiation therapy treatments for another type of cancer
• Having changes in certain genes, including BRCA1, BRCA2, PALB2, CHEK2, and PTEN

If you are interested in learning more about your own risk of developing inflammatory breast cancer, talk to your doctor.

Symptoms of Inflammatory Breast Cancer

Many other types of breast cancer lead to a distinctive lump within the breast tissue. However, people with IBC don’t usually have a lump. Instead, they have a different set of signs and symptoms. As this cancer progresses, one breast starts to look very different over a very short period of time. In the affected breast, you may notice:

• Swelling or heaviness, making one breast suddenly seem larger than the other
• Red, purple, or dark-colored skin covering all or part of the breast
• Warmth or heat
• Pain or tenderness
• Itching or burning sensations
• Peau d’orange (the skin of the breast starts to look pitted or dimpled, like the surface of an orange peel)
• The nipple looks flat, becomes inverted, or has discharge

Some people with inflammatory breast cancer also notice swollen lymph nodes. This may feel like a hard lump in the armpit or near the collarbone.

Having these symptoms doesn’t necessarily mean that you have cancer. They are also frequently caused by other conditions, such as mastitis (inflammation or an infection of the breast tissue). However, you should see a doctor right away if you notice that one breast suddenly starts to look different or is undergoing skin changes.

Inflammatory Beast Cancer Tests and Diagnosis

If you are experiencing symptoms of IBC, your doctor will likely have you undergo imaging tests. These tests may include a mammogram, ultrasound, or MRI. These imaging tests can help the doctor see inside of the breast and identify any problems, such as a tumor.

Unlike other types of breast cancer, IBC often doesn’t form lumps, so it may be difficult to diagnose with standard imaging tests. Doctors need to perform a biopsy in order to confirm whether a person has IBC. During a biopsy, the doctor removes a small piece of tissue. This tissue is then studied under a microscope to determine whether cancer cells are present. Your doctor may use a skin punch biopsy, in which a tiny bit of skin and underlying tissue is taken. Alternatively, your doctor may perform a core needle biopsy, in which ultrasound imaging is used to guide the needle to a deeper section of breast tissue in order to remove cells there.

If you receive a diagnosis of inflammatory breast cancer, your doctor may recommend additional imaging tests to see whether cancer cells have spread to other parts of the body. These other imaging tests may include positron emission tomography (PET), computed tomography (CT), or a combination.

Types of Inflammatory Breast Cancer

When diagnosing breast cancer, including IBC, doctors will determine the molecular subtype of the breast cancer. To do this, doctors perform tests on biopsy samples to see which proteins are found on the surface of cancer cells. This information helps doctors understand how breast cancer cells grow, know which treatments may work best, and estimate prognosis.

Doctors will test for proteins called hormone receptors, including estrogen receptor (ER) and progesterone receptors (PR), and will determine whether cells contain a protein called HER2. If cells lack ER, PR, and HER2, doctors say that the cancer is “triple negative.” Triple negative breast cancers tend to grow more quickly and are harder to treat. Many people with IBC have triple negative cancer.

Staging Inflammatory Breast Cancer

Tests can also help your doctor determine your cancer stage. The cancer’s stage describes how far cancer cells have spread. Inflammatory breast cancer is usually considered at least stage 3 (sometimes written in Roman numerals as stage III) — it has spread to nearby tissues — when it's first diagnosed. Doctors stage IBC as follows:

• Stage 3B — IBC cells have spread to the skin or chest wall (the muscles and bones of the chest) and have possibly spread to nearby lymph nodes.
• Stage 3C — IBC cells have spread to other tissues in the area as well as at least 10 nearby lymph nodes.
• Stage 4 (also called metastatic breast cancer) — IBC cells have spread to other parts of the body, such as the lungs, liver, or bones.

Treatment of Inflammatory Breast Cancer

Doctors usually recommend an aggressive treatment plan for IBC. Treatment for stage 3 IBC often starts with chemotherapy or other cancer-killing drugs to help shrink the cancer. Next, a person with IBC often receives surgery to remove the breast tissue (mastectomy) and nearby lymph nodes. After surgery, a person may receive radiation therapy or additional drug treatments.

Chemotherapy, targeted therapy, and hormone therapy are the main treatments used for stage 4 IBC. Surgery and radiation therapy may be used, but these treatment options are less common for metastatic disease.

Chemotherapy, Targeted Therapy, and Hormone Therapy

Chemotherapy drugs are often liquids given through an intravenous tube or orally through capsules or pills. These drugs travel throughout the body killing cancer cells. When chemotherapy is given before surgery, doctors may call it “neoadjuvant chemotherapy.”

Some cases of IBC are HER2-positive. These cancers are sensitive to certain targeted therapy drugs such as Herceptin (trastuzumab), Perjeta (pertuzumab), and other drugs that target HER2. Targeted therapy drugs can recognize and block certain proteins found on the surface of cancer cells, thereby killing the cells.

Another drug option may be hormonal therapy. If IBC cells have ER or PR proteins, hormone therapy can stop or slow down cancer cells’ growth.

Surgery

If chemotherapy successfully shrinks the cancer, a person with IBC may have surgery. Doctors usually recommend a mastectomy, in which all of the breast tissue is removed. The surgeon may also perform an axillary dissection to take out nearby lymph nodes. After surgery, a person with IBC may have radiation treatments, or they go through additional rounds of chemotherapy, targeted therapy, or hormone therapy.

Radiation Therapy

During radiation therapy, high-energy particles or beams are delivered to the breast and surrounding area. Most people with IBC receive radiation therapy because it is difficult to remove all of the cancer cells during surgery. A person will usually undergo radiation therapy five days per week for five to six weeks.

Clinical Trials

Many experts recommend that people with IBC look into clinical trials. During these studies, researchers will test new drugs, doses, or treatment protocols. Clinical trials may give people with IBC a chance to receive potentially more active treatments and help others with breast cancer.

What Is the Prognosis for IBC?

Inflammatory breast cancer often leads to a poor prognosis (outlook). To learn more about your individual prognosis, talk to your doctor, who is familiar with your overall health and your personal health history.

When estimating prognosis, doctors use studies consisting of large groups of people who were all diagnosed with IBC. Researchers may measure prognosis in several ways. One possibility is the five-year relative survival rate. This number tells you how many people with IBC are likely to survive for five years, compared to those without IBC. Among all stages of IBC, women with this cancer are 41 percent as likely to live for five years or more than are women without IBC.

A person’s prognosis depends on what stage of cancer they have. The five-year relative survival rate is 56 percent for women with stage 3 IBC and 19 percent for women with stage 4 IBC.

A slightly different way of measuring prognosis is to use a number called the overall survival rate. This number tells you how many people diagnosed with a condition are alive after a certain amount of time. Various studies have looked at large groups of people with IBC and found the following overall survival statistics: About 71 percent of people with nonmetastatic IBC live for at least five years after being diagnosed. About 39 percent of people with metastatic IBC live for two years or more after diagnosis. Half of those with metastatic disease live for 18 months or more.

Prognostic Factors

Certain characteristics can raise or lower a person’s chances of having a good prognosis. These are called prognostic factors.

Cancer stage is one prognostic factor. People with breast cancer that has begun to spread around the body have a worse prognosis than those who only have cancer cells within the breast.

Additionally, a cancer’s molecular subtype affects outlook. People with IBC who have triple negative breast cancer have a worse prognosis than people with other subtypes. Those with HER2-positive breast cancer have a better outlook.

Other factors that can affect prognosis include a person’s age, whether they have other health conditions, tumor grade (how abnormal cancer cells look and how fast they grow), and the treatments a person receives.

Talk With Others Who Understand

MyBCTeam is the social network for people with breast cancer and their loved ones. On MyBCTeam, more than 53,000 members come together to ask questions, give advice, and share their stories with others who understand life with breast cancer.

Are you living with inflammatory breast cancer? Share your experiences in the comments below, or start a conversation by posting on MyBCTeam.