Inflammatory breast cancer

Inflammatory breast cancer, or IBC, is an aggressive type of invasive breast cancer that usually doesn’t form a lump. Instead, typical symptoms include breast swelling, color changes, skin thickening, and dimpled skin.

Not all doctors are trained to recognize the symptoms as inflammatory breast cancer. Breast lumps aren’t common with IBC, so a mammogram is unlikely to pick it up. As a result, doctors often mistake inflammatory breast cancer for other illnesses. But because IBC spreads quickly, it’s important to get an accurate diagnosis and treatment as soon as possible.

It can be shocking to receive an IBC diagnosis — and even more upsetting if diagnosis is delayed when doctors don’t recognize IBC symptoms. LBBC is here for you. Below, we’ll explain how IBC is diagnosed and treated, how to find an IBC specialist, ways to get support, and more.

Who gets inflammatory breast cancer?

Inflammatory breast cancer makes up about 1 to 5% of all breast cancers diagnosed in the U.S. each year.

IBC can affect women of all ages, and it can happen in men. However, diagnosis is more common in:

• Younger women: it’s often diagnosed in people under age 40, with an average age of 59 at diagnosis
• Black women

Other risk factors can include:

• Having a first-degree relative with a history of breast cancer
• Having highly dense breasts: There is little to no fatty tissue in the breasts.

Otherwise, risk factors are similar to those for other types of breast cancer. Learn more about general risk factors.

Inflammatory breast cancer symptoms

Symptoms of inflammatory breast cancer can include:

• Breast swelling
• Red patches or other skin color changes in the breast
• Breast pain
• Sensations of heat
• Thickening of the skin
• Peau d’orange: the breast skin looks pitted or dimpled, like an orange peel
• Inverted nipple: the nipple appears turned inside out

Instead of forming a lump, IBC grows in sheets or nest-like patterns. It travels through the lymph vessel system, a network of tubes that helps to balance fluid in the body and protect against infection. IBC forms clumps of cancer cells that clog lymph vessels in the breast. Lymph fluid can’t drain as it normally would.

Trapped in the lymph nodes and vessels, these cancer cell clusters cause swelling, redness, pain, and nipple inversion. Often, symptoms appear quickly, over several days or weeks.

Diagnosing inflammatory breast cancer

Doctors often mistake IBC for a rash or a breast infection called mastitis. This can lead to delays in diagnosis.

If your doctor prescribes an antibiotic cream or pill and the symptoms don’t get better within a few days, you should be examined for inflammatory breast cancer. Call your doctor and ask for a referral to a breast specialist. This could be a surgeon or medical oncologist who has experience with IBC.

To diagnose IBC, the doctor will perform a physical breast exam and examine visible symptoms, such as growing breast size, redness, and skin changes. They also will order a series of tests. These can include:

• Imaging tests, such as mammogram, ultrasound, and breast MRI
• Biopsy to remove a sample of the skin and/or breast tissue for testing.
  • A punch biopsy of the skin is often done to check for IBC. This type of biopsy removes a small circular piece of the breast skin.
  • A needle biopsy may be done to remove a sample of the breast tissue.
• Blood tests, such as complete blood count

Tests on the cancer tissue

After a biopsy confirms that inflammatory breast cancer is present, the tissue will be tested for other features that can help guide treatment planning. These include:

• Hormone receptors, to find out if the cancer has receptor proteins for the hormones estrogen and/or progesterone. If it does, the cancer is hormone receptor-positive (HR+), and hormones are fueling its growth.
• ​​​HER2 receptors, which receive signals that tell the cancer cells to grow and divide out of control. The cancer may be described as HER2-positive, HER2-low, HER2-ultralow, or HER2-negative.
• Grade, a measure of how abnormal the cells appear and how quickly they are growing.

Although inflammatory breast cancer can have any hormone receptor or HER2 status, it’s more likely than other types of breast cancer to be:

• Hormone receptor-negative
• HER2-positive
• Triple-negative (tests negative for estrogen and progesterone receptors and HER2)

These and other features of the IBC will be described in a pathology report that you will discuss with your healthcare team.

If the cancer is found to be stage IV (metastatic), additional tests will be done on the cancer tissue or on blood samples. For example, biomarker testing, also called genomic testing, uses tissue or blood to detect gene changes, proteins, or other biological features of the cancer. This information can help match the cancer to targeted therapy or immunotherapy.

Additional imaging tests

Because inflammatory breast cancer advances quickly, it’s more likely than other types of breast cancer to move beyond the chest area to other parts of the body (stage IV cancer). Your doctor may order additional imaging tests to check for signs of cancer elsewhere.

These can include:

• PET scans
• CT scans
• Bone scans

Imaging that combines a PET scan and CT scan, called PET/CT, is often recommended for inflammatory breast cancer.

Genetic testing for inherited mutations

Certain inherited genetic mutations, or abnormal gene changes, increase breast cancer risk and can be passed through families. If your doctor does not discuss genetic testing with you, ask to be referred to a genetic counselor to learn more about genetic testing for inherited mutations.

The American Society of Clinical Oncology recommends that all newly diagnosed patients under age 65 be tested for mutations in the BRCA1 and BRCA2 genes. It is also recommended for:

• Diagnosed people over 65, based on personal and family history
• People who may be eligible for a specific type of targeted therapy (called PARP inhibitors)

Genetic test results can help you and your doctor make treatment decisions. They also can help your family members understand whether they could be at increased risk.

Based on medical and family history, doctors also may recommend testing for mutations in genes such as PALB2, TP53, PTEN, STK11, and CDH1. A genetic counselor can help you understand how the results could be helpful to you and your family.

Visit Genetic testing for inherited mutations to learn more.

Treatment for inflammatory breast cancer

Standard treatment for IBC is often different than it is for people with other types of invasive breast cancer. Inflammatory breast cancer is usually diagnosed at stage III (early-stage) or IV (metastatic) and spreads quickly.

The goal is to remove all local disease in the breast and also destroy any cancer cells that might have moved throughout the chest area or into other areas of the body.

The standard of care is often called trimodal care or trimodality therapy. It includes three forms of treatment:

• Chemotherapy, sometimes in combination with targeted therapy, before surgery (this is called neoadjuvant therapy)
• Surgery: Mastectomy to remove all of the breast tissue and lymph node surgery to remove most or all of the underarm lymph nodes
• Radiation therapy

Studies have shown that women with IBC who get trimodality treatment have better outcomes than those who do not. However, some treatment teams aren’t experienced with it. Research suggests that not all women with IBC are receiving this treatment approach.

If you’re weighing options, be sure to discuss trimodality therapy with your care team. If they’re not experienced with IBC, seek out a second ​​opinion from another doctor who is. You also can ask your team if they’d be willing to consult with a doctor who sees more cases of inflammatory breast cancer.

Treatment for early-stage IBC

For early-stage IBC, standard treatment starts with ​​​​neoadjuvant therapy, or treatment before surgery.

Neoadjuvant treatment for early-stage IBC

Neoadjuvant chemotherapy can help shrink the cancer so it is easier to remove during surgery. Additional treatments are often given along with chemotherapy:

• If the cancer is HER2-positive, you will likely receive neoadjuvant treatment with trastuzumab (Herceptin), pertuzumab (Perjeta), or another HER2-targeted therapy. These medicines can cause heart problems when given along with specific chemotherapies, so your healthcare team may give the treatments one after the other.
• If the cancer is triple-negative, the immunotherapy pembrolizumab (Keytruda) may be given before surgery. This treatment often continues after surgery.

After neoadjuvant therapy, a radiologist may do imaging tests to see whether the medicines worked against the cancer. If they weren’t effective, your doctor may recommend trying a different chemotherapy medicine or giving radiation therapy.

Surgery for early-stage IBC

Surgery for early-stage IBC includes:

• Mastectomy to remove all of the breast tissue, including the nipple
• Surgery to remove most or all of the lymph nodes

Because IBC can spread quickly, your surgeon may also need to remove:

• The lining over the underlying chest muscles
• The smaller chest muscle (pectoralis minor)

Your surgeon will aim to remove all areas of cancer with clear margins. This means there is a rim of healthy, cancer-free tissue surrounding the tumor(s). More surgery will be needed if the margins aren’t clear.

If a person chooses breast reconstruction after mastectomy, it must be delayed until after other treatments have been given.

Adjuvant treatment for early-stage IBC

Adjuvant treatment (treatment after surgery) for early-stage IBC will likely include radiation therapy. Radiation targets the chest wall where the breast was removed. The goal is to reduce the risk of IBC returning in the chest area.

Adjuvant treatment may also include the following therapies to reduce the risk of recurrence:

• Additional chemotherapy may be given in some cases.
• If the cancer is hormone receptor-positive, hormonal therapy may be given after ​​surgery.
• If the cancer is HER2-positive, HER2-targeted therapy may continue after surgery.
• If the cancer is triple-negative and you had neoadjuvant treatment with pembrolizumab, this treatment will likely continue after surgery.
• If you have an inherited BRCA genetic mutation and the cancer is triple-negative or HER2-negative, olaparib (Lynparza), a PARP inhibitor targeted therapy, may be an option.

Your care team will work with you to decide on the best combination of treatments for your situation.

Treatment for metastatic IBC

If the IBC is metastatic, or stage IV, it means the cancer has moved beyond the breast and surrounding lymph nodes to other parts of the body, such as the bones, lung, liver, or brain.

Treatment for metastatic IBC may include:

• Chemotherapy
• Targeted therapy
• Immunotherapy
• Hormonal therapy

The use of targeted therapy, immunotherapy, or hormonal therapy depends on the specific features of the cancer, such as whether the cancer has hormone receptors, HER2 receptors, or other biomarkers.

Surgery and radiation therapy may also be used in certain situations to control the cancer and/or for symptom relief. Learn more about treatment for metastatic breast cancer.

Breast reconstruction after inflammatory breast cancer

Some women choose to have surgery to rebuild the breast, called breast reconstruction, after mastectomy. Others decide to go flat.

With inflammatory breast cancer, it’s not possible to do immediate breast reconstruction at the time of mastectomy. This is because:

• Your surgeon needs to remove as much skin as possible. Performing reconstruction right away would require that breast skin be left ​​​​behind.
• Treatment includes radiation therapy. The chest area needs at least 6 months or more time to heal before reconstruction can be done.
• Treatment may sometimes include additional chemotherapy and/or other treatments after surgery, which should not be delayed due to breast reconstruction procedures.

In many cases, it is possible for women with IBC to have delayed breast reconstruction at a later date. Tissue reconstruction, also called autologous flap reconstruction, is the preferred approach. It uses a section of skin, fat, and blood vessels from another part of the body to reconstruct the breast.

Breast reconstruction with implants may not be recommended because of the damage and scarring caused by radiation therapy. These side effects can lead to complications with healing and make it more difficult to achieve a good implant reconstruction result. Also, after mastectomy, surgeons need to place an expander under the skin to make room for a future implant. This can be difficult when all of the breast skin has to be ​​removed. Still, in some cases, women with IBC do have implant reconstruction.

If you’re interested in breast reconstruction, let your doctor know as you are working together to plan your treatment. Visit Breast reconstruction to learn more.

Protecting your fertility if you want to have children

If you’re premenopausal (still having periods) and want to have biological children, it’s important to talk with your doctor about fertility preservation before starting treatment if possible.

Some breast cancer treatments, such as chemotherapy, hormonal therapy, and immunotherapy, can interfere with fertility or pregnancy timing. However, there are still options for having a baby. You can ask your doctor to refer you to a fertility specialist who can help you understand those options.

Learn more about fertility treatment options.

How to find an IBC specialist

Many doctors aren’t that familiar with inflammatory breast cancer. Here are some tips for finding a doctor with experience treating IBC:

• If possible, seek out treatment at an academic medical center with programs and research focused on IBC. Usually, this means the medical center is affiliated with a medical college or university. These centers often conduct research and clinical trials.
• If you are looking online for breast cancer providers, check their list of specialties to see if inflammatory breast cancer is listed.
• Ask any team you meet with whether they have treated people with IBC in the past.
• If specialists aren’t available in your area, ask your team if they are willing to consult with doctors who have more experience with IBC.

Researchers are working toward a better understanding of IBC and how to treat it. If you’re interested in helping research teams study new treatments and learn more about IBC, here are some options:

• Taking part in a clinical trial means participating in a study that looks at how effective new treatment approaches are with specific cancers. Clinical trials offer access to new treatments that aren’t widely available and might work better than standard treatments. Trials also help researchers develop better treatments for people diagnosed in the future.
• Donating a breast tissue sample and providing information about your diagnosis to the IBC Research Foundation BioBank or to clinical trials can help researchers learn more about IBC. Ask your healthcare team for more information.

Resources for information and ​​support

Inflammatory breast cancer is rare, making up 1 to 5% of breast cancers. An IBC diagnosis can feel frightening and frustrating — but it’s important to know that you’re not alone. Visit LBBC’s Flourish, a free online community where you may be able to connect with others who have IBC.

Here are some additional resources specifically for people diagnosed with IBC:

• The IBC Network Foundation, which offers education and support, a newsletter, and Zoom chats
• Inflammatory Breast Cancer Research Foundation, which supports research and features patient stories
• Dana-Farber Cancer Institute, Inflammatory Breast Cancer Support and Education

For emotional support resources, visit these LBBC pages:

• Emotional health
• Managing emotional side effects
• Where to find breast cancer support
• Help for anxiety, including ways to take care of yourself
• Help for depression
• Seeing a mental health professional
• Emotional support for LGBTQ+ people affected by breast cancer