An Overview of Inflammatory Breast Cancer (IBC)

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

Compared to other forms of breast cancer, inflammatory breast cancer tends to strike younger women and is more common in black women than white women. Men who are diagnosed with IBC are older on average than female patients.

IBC is often mistaken for other conditions, so it's important to see a doctor if you're experiencing any of its symptoms.

Symptoms

As IBC grows, it blocks lymph vessels and blood vessels in the breast. Signs and symptoms can develop quickly, sometimes seemingly overnight, or over a few weeks or months, and may include:

  • Redness and/or a rash on the skin: There may be areas that are skin color, pink, red, or very dark with a blue or purple tinge. This redness is usually fairly extensive, covering a third of the breast or more.
  • Sudden increase in breast size (as much as a cup size in a few days)
  • Skin dimpling akin to an orange peel (called peau d'orange)
  • Constantly itching breast skin
  • Breast heaviness (one more than the other)
  • Firmness or hardening of one breast
  • Warmth of one breast
  • 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

With IBC, many people may at first think they have a benign skin rash such as eczema or an infection such as mastitis. These cancers may not show up on a mammogram, so being suspicious of symptoms, should they appear, is critical.

Causes and Risk Factors

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

  • Being female: Women are more likely to be diagnosed with IBC than men, but men also can develop this type of cancer. 
  • Age: IBC is more common in younger women than older women.
  • Ethnicity: Black women have a higher risk for IBC than do white women.
  • Weight: Women who are overweight or obese have a slightly increased risk, but many women of healthy weight develop the disease as well.

Diagnosis

IBC can be challenging to identify, but there is a set of criteria to help doctors reach the official diagnosis:

  • Symptoms, such as the above, that appear rapidly. A mass may or may not be present.
  • Symptoms affect a third or more of the breast.
  • Duration of symptoms is less than six months.
  • A biopsy shows invasive cancer (see below).

Breast Exam

Your doctor 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

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

Imaging Studies

After taking a careful history and doing a physical exam, your doctor will likely order imaging studies or a do a breast biopsy to further understand your symptoms. These studies cannot only help diagnose an inflammatory breast cancer, but help rule out conditions that appear similar, such as mastitis.

Tests that may be done include:

  • Mammogram: As noted, a mammogram may be negative with IBC, but may also show evidence of skin thickening, a tumor, or increased breast density.
  • Ultrasound: Ultrasound may not be as helpful with IBC if a discrete mass is not present, but may be helpful in evaluating axillary lymph nodes (armpit nodes).
  • Computed tomography (CT) scan: CT may be helpful in determining if the cancer has spread to distant sites.
  • Magnetic resonance imaging (MRI): An MRI can provide information about soft tissues and may detect IBC that is not seen on a mammogram.
  • Bone scan: A bone scan is often done to look for the spread of cancer to bones.
  • Positron emission tomography (PET) scan: A PET scan is a sensitive test for detecting areas of active cancer growth in the body. It is usually done more for staging than for diagnosis and can be helpful in identifying possible metastases to lymph nodes and other parts of the body.

Biopsy

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

Most inflammatory breast cancers are diagnosed via biopsy as invasive ductal carcinomas.

Staging

Inflammatory breast cancer commonly grows in nests or sheets, not in lumps. It spreads through the body primarily via the lymph system. Early on, these tumors may appear to be slow-growing, low-grade tumors, but once the skin on the breast is inflamed, it can metastasize rapidly.

Unlike the breast cancer stages that most people are familiar with (stages 1 through 4), IBC is classified either as stage 3 or stage 4. Stage 3 cancers are larger than 5 centimeters in diameter and have spread to at least one lymph node, but not to other regions of the body. Stage 4 cancers are similar to stage 3B, but the cancer has also spread to distant regions of the body.

Treatment

Inflammatory breast cancer is aggressive. Therefore, it is usually treated aggressively with a combination of therapies to reduce the risk of recurrence (for stage 3).

Neoadjuvant Chemotherapy

Neoadjuvant chemotherapy refers to chemotherapy that is administered prior to surgery. A combination of drugs is usually given in cycles for four to six months.

Surgery

The most common surgery is modified radical mastectomy, which is similar to a mastectomy for other types of breast cancer. With IBC, however, one of the chest muscles (pectoral minor) may be removed.

Most lymph nodes are also removed, not just some, as with a sentinel node biopsy.

If women desire reconstructive surgery, it is usually delayed until at least six months after radiation therapy has been completed.

Radiation Therapy

Radiation therapy is usually used after a mastectomy to treat the chest wall and any remaining lymph nodes.

Targeted Therapies

Many inflammatory breast cancers are HER2 positive, so treatment with HER2-targeted therapies can be very effective in controlling the tumor. These drugs are usually given along with the other treatments after a diagnosis of IBC.

Clinical Trials

There are a number of clinical trials in progress for inflammatory breast cancer that are looking at combinations of the treatments above as well as newer treatments, such as immunotherapy.

Most inflammatory breast cancers are estrogen receptor and progesterone receptor negative, so hormonal therapy with tamoxifen or aromatase inhibitors isn't commonly used.

Recurrence

IBC has a higher risk of recurrence than some other forms of breast cancer. If recurrence does occur, treatment is available and may include HER2-targeted therapies, chemotherapy, hormonal therapy, or other treatments in clinical trials.

Recurrence is possible at any time, whether it is a months from the final treatment or years down the road. The recurrence can be local, either in the previously treated breast or near the mastectomy scar, in the lymph nodes or collar bone on the same side as the previously affected breast, or somewhere else in the body. The most common sites for recurrence are the lymph nodes, bones, liver, and lungs.

The good news is that most breast cancers don’t return. However, doctors cannot predict with certainty which tumors can result in a recurrence. Fortunately, they do have some idea which factors may increase the risk of one:

  • Tumor size: Larger tumors are more likely to recur.
  • Age: The risk is greater in a person diagnosed at 35 years of age or younger.
  • Having a lumpectomy without radiation
  • Lymph node involvement
  • Nuclear grade: Some cancer cells are more aggressive and prone to recurrence.

The highest risk of recurrence occurs in the first few years following treatment and person’s risk is reduced after they have been cancer-free for five years. One 2018 review looked at data from people with triple-negative breast cancer. The results indicated if a person survived for five years following treatment, there was a low probability of it recurring in the next year. IBC recurrences tend to recur earlier because it is more aggressive and progresses much quicker than other types of breast cancer.

Your doctor will want you to come in periodically to check for recurrences. The timetable for how often is determined based upon your unique situation, but for most types of breast cancer, general physical exam follow-ups are every three to six months after diagnosis, and then yearly thereafter (including annual mammograms).

Because of the potential for recurrence with IBC is high, make sure you are performing monthly breast self-exams and paying attention to symptoms of recurrence. 

A Word From Verywell

It's important to remember that each person and every cancer is different. While treatment for IBC can be challenging, know that there are long-term survivors of this disease. In addition, newer treatments often have fewer side effects than traditional chemotherapy drugs.

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