Inflammatory Breast Cancer Symptoms and Treatment

Inflammatory breast cancer (IBC) is an uncommon and aggressive type of breast cancer. It can cause the breast to appear red and swollen, giving the appearance of inflammation. In the United States, diagnoses of IBC accounts for 1% to 5% of all breast cancer cases.

Compared to other types of breast cancer, IBC tends to strike younger women and is more common in Black women than White women. It's a rare diagnosis in males. IBC is often mistaken for other conditions. It's important to talk with a healthcare provider if you experience symptoms.

Symptoms

IBC is a fast-growing cancer that can block lymph and blood vessels in the breast. As a result, symptoms develop quickly, sometimes over weeks or months. IBC may or may not contain a solid tumor that you can feel.

The symptoms can be similar to those of other conditions, so it's essential to watch for physical changes and describe them to your healthcare provider. Common symptoms of IBC include:

• Redness and/or a rash on the skin: There may be areas of the breast that are pink, red, or bluish (like a bruise). This redness is usually fairly extensive and can cover one-third of the breast or more. The skin also may itch.
• Sudden increase in breast size (as much as a cup size in a few days)
• Skin dimpling similar to an orange peel (called peau d'orange)
• Breast heaviness (one side more than the other)
• Hardness or burning sensations in the breast
• Feeling that one breast is warmer than the other
• Breast pain that is not related to your menstrual cycle
• Nipple retraction or other nipple changes
• Swollen lymph nodes under the arms or above the collarbone

Causes and Risk Factors

Researchers aren't sure what causes IBC specifically, but some risk factors have been identified, including:

• Gender: Though IBC affects both males and females, men are far less likely to be diagnosed.
• Age: IBC is more common in younger people (in their 40s or 50s).
• Ethnicity: Black women are at a greater risk for developing IBC than White women.
• Weight: People who are overweight or obese have a slightly increased risk of IBC, but it can impact people of average weight as well.

Diagnosis

IBC can be challenging to identify, but there is a set of criteria to help healthcare providers reach a diagnosis, such as:

• Symptoms come on quickly. A mass may or may not be present.
• Symptoms affect one-third or more of the breast.
• Duration of symptoms is less than six months.
• A biopsy (removing tissue to be examined in a lab) shows invasive cancer.

Genetic changes are often used in assessing cancer. A study at the Dana-Farber Cancer Institute compared genetic changes in IBC with other breast cancers. It finds that different markers aren't really present overall, with the possible exception of TP53 changes more common in IBC.

The TP53 mutation occurred in 72% of the 140 IBC cases included in the study of 2,457 metastatic (advanced) breast cancers. More research is needed to confirm and understand the results.

Breast Exam

Your healthcare provider will perform a clinical breast exam, including a visual check of your breast. This involves looking for changes in skin color that may be caused by cancer cells blocking the lymph nodes and vessels in your breast skin. If your breast is swollen, it may be caused by fluid buildup, a condition called edema. 

If your breast skin is ridged, pitted, bumpy, or resembles an orange peel, that will also be noted. Your healthcare provider will also check the lymph nodes in your armpits. 

Imaging Studies

After taking a careful medical history and doing a physical exam, your healthcare provider will likely order imaging studies or perform a breast biopsy to understand your symptoms further. These studies help diagnose IBC and help rule out conditions, such as mastitis, that can cause similar symptoms.

These tests include:

• Mammogram: A mammogram may be negative because IBC does not always come with a solid tumor. However, this test can show skin thickening or increased breast density, both signs of potential IBC.
• Ultrasound: Ultrasound may not be as helpful with IBC if a mass is not present but may help evaluate axillary lymph nodes (armpit nodes).
• Computed tomography (CT) scan: CT may help determine if cancer has spread to other parts of the body.
• Magnetic resonance imaging (MRI): An MRI can provide information about soft tissues and may detect IBC that is not visualized on a mammogram.
• Bone scan: A bone scan is often done to look for the spread of cancer to the bones.
• Positron-emission tomography (PET) scan: A PET scan is a sensitive test that detects areas of active cancer growth in the body. It is usually done more for cancer staging than for diagnosis and can help identify metastases (spread) to lymph nodes and other body parts.

Biopsy

If a mass is noted, a breast biopsy may be performed. If a mass is not present, a skin biopsy will be done on the abnormal area of the affected breast.

There may be different approaches. For example, experts working in partnership with Susan G. Komen and the Inflammatory Breast Cancer Research Foundation have proposed a point-based assessment. It would indicate a skin biopsy is needed at a certain point threshold, regardless of whether there's a mass or not.

Staging

IBC does not always present with a lump like other cancers. Instead, it grows in sheets (sometimes called "nests") and can spread through the body primarily via the lymphatic system. With IBC, the diagnosis itself means there's been metastasis (spread) into other tissues.

As a result, IBC is classified either as stage 3 or stage 4, depending on how far it has spread in the body. Stage 3 cancers have spread to at least one lymph node but not to other body regions. Stage 4 cancer is similar to stage 3B, but the cancer has spread to distant body areas.

Treatment

Inflammatory breast cancer is aggressive. Therefore, it is usually treated with a combination of therapies (sometimes called a "multimodal approach") to reduce the risk of recurrence. These treatments include:

• Neoadjuvant chemotherapy: This refers to chemotherapy that is administered prior to surgery. A combination of drugs is usually given in cycles for four to six months, depending on how quickly the cancer is growing. In some cases, patients may receive additional chemotherapy after surgery (called adjuvant chemotherapy).
• Surgery: The most common surgery is modified radical mastectomy (removing the entire breast), which is similar to a mastectomy for other types of breast cancer. With IBC, however, the lining of the chest muscles is also removed. Sometimes, one of the chest muscles (pectoral minor) may be removed. In addition, most lymph nodes are also excised.
  
• Radiation therapy: This is usually performed after a mastectomy to treat the chest wall and remaining lymph nodes. Reconstructive surgery (plastic surgery to restore the appearance of the breast) is usually delayed until at least six months after completion of radiation therapy.
• Targeted and hormone therapies: Many IBC cases are HER2 positive (a protein that makes cancer grow), so treatment can include HER2-targeted therapies. If the cancer is sensitive to estrogen, hormone therapy may also be an option. IBC is typically estrogen receptor and progesterone receptor negative, so hormonal therapy with tamoxifen or aromatase inhibitors isn't commonly used.
• Clinical trials: There are a number of clinical trials in progress for inflammatory breast cancer that are evaluating the combination of the treatments above as well as newer treatments, such as immunotherapy (treatment that uses your body's immune system to help fight cancer).

Recurrence

IBC is more aggressive than some forms of breast cancer and has a high rate of recurrence. If recurrence does occur, treatment is available and may include HER2-targeted therapies, chemotherapy, or hormonal therapy. In addition, other medicines may be available in clinical trials.

Recurrence is possible at any time, whether months after treatment or years down the road, including:

• If IBC recurs in the area of a reconstructed breast or near the mastectomy scar, it is considered a local recurrence.
• Regional recurrence is found in the lymph nodes or near the collarbone on the same side as the previously affected breast, and a distant recurrence is found elsewhere in the body. 
• The most common sites for recurrence are the lymph nodes, bones, liver, and lungs.

Healthcare providers cannot predict which tumors will result in a recurrence with certainty. After treatment, careful monitoring will continue.

Summary

Inflammatory breast cancer is an aggressive type with different symptoms than other breast cancers, including redness, rash, or dimpling. Once a biopsy and imaging are complete, IBC can be staged. Treatment for this type of breast cancer can include chemotherapy, hormonal therapy, or targeted therapy. You may also need surgery and radiation to treat IBC.

Many clinical trials are looking into better treatment options for IBC. While treatment for IBC can be challenging, there are long-term IBC survivors and treatment options continue to evolve.

Breast Cancer Discussion Guide

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