Inflammatory Breast Cancer : Clinical Features, Diagnosis and Treatment

Last Updated on March 18, 2020

Inflammatory breast cancer is a rare but extremely aggressive type of breast cancer. In this disease, the cancer cells block the lymph vessels present in the skin of the breast. It is called “inflammatory” because the breast appears red, swollen and inflamed.

Symptoms of inflammatory breast cancer can look very similar to breast infection ( mastitis and breast abscess) which is a much more common cause of breast swelling and redness. This makes it important to seek urgent medical advice if a woman notices any change on the breast skin or signs of breast infection.

Inflammatory breast cancer progresses rapidly, often in a matter of weeks or months. At diagnosis, inflammatory breast cancer is either stage III or IV disease, depending on whether cancer cells have spread only to nearby lymph nodes or to other tissues as well.

Inflammatory breast cancer is a rare type of breast cancer accounting for about 1 to 5 percent of all breast cancers.

Risk factors

Women of African-American origin
Obese women

Signs and Symptoms of Inflammatory Breast Cancer

Inflammatory breast cancer doesn’t usually present as a breast lump. This is in contrast to other forms of breast cancer whose most common symptom is the presence of a breast lump.

The most common symptom of inflammatory breast cancer is swelling and redness that affects at least one-third of the breast. The skin of the breast may show discoloration, having a red, purple, pink or bruised appearance. The skin may have a pitted appearance resembling an orange peel (called peau d’orange).
These symptoms occur due to the blockage of lymph vessels by cancer cells which prevents the normal flow of lymph through the breast tissue.
The affected part of the breast may feel unusually warm.
The involved breast may be tender, painful or have a dull aching pain.
There may be a rapid increase in breast size or a sensation of heaviness.
There may be flattening or inversion (turning inward) of the nipple.
Enlarged lymph nodes may be present in the axilla and above or below the collarbone

Special Features

It doesn’t usually produce a breast lump. This makes it difficult to diagnose this entity.
It tends to occur in younger women (less than 40 years of age)
It is more aggressive. It has a much greater tendency to grow and spread than other types of breast cancer. It is also more likely to recur after treatment than other types of breast cancer.
Since the cancer cells have already grown into the skin, it is usually at an advanced stage (at least stage III) at the time of diagnosis.
It has a worse prognosis as compared to other breast cancers. This occurs due to delay in diagnosis and advanced stage at the time of presentation.
It accounts for about 1% to 5% of all breast cancer cases. However, because of its aggressive nature, it accounts for 10% of all breast cancer deaths.

Inflammatory Breast Cancer vs. Breast Infection

Symptoms of inflammatory breast cancer are similar to those of mastitis.

Mastitis is an inflammation of the breast due to an underlying infection. It causes the breasts to become red, swollen and painful. Although it can occur in any woman, it most commonly occurs in women who are breastfeeding.

In breastfeeding women, the infection occurs due to a blocked milk duct with bacteria entering the skin through a crack or break around the nipple. In non-breastfeeding women, mastitis most often occurs when the breast becomes infected as a result of damage to the nipple, such as a cracked or sore nipple, or a nipple piercing.

Mastitis may also result in fever, headache, and/or nipple discharge. These symptoms are not usually seen in inflammatory breast cancer.

Mastitis is treated by anti-inflammatory drugs and antibiotics. If the symptoms do not resolve within a couple of days even after proper treatment, the patient must be investigated further to rule out underlying inflammatory breast cancer.

Diagnosis

Inflammatory breast cancer is diagnosed by a combination of imaging tests and biopsy.

Mammogram

It may reveal whether the affected breast is denser or if the affected skin is thicker than the other breast.

Breast ultrasound

During pregnancy and lactation, ultrasound is considered superior to mammography because the hormone-induced changes in breast tissue cause an increase in the density of breast tissue making interpretation of mammograms difficult.

Breast MRI (Magnetic Resonance Imaging)

MRI can be used to diagnose breast cancer in select high-risk patients, in cases of dense breast tissue or when findings of mammogram and ultrasound are not conclusive.

Nipple discharge cytology

The discharge from the nipple can be smeared on a glass slide and examined under a microscope to look for the presence of malignant cells.

Biopsy

It is a small surgical procedure carried out under local anesthesia. A small piece of the affected breast and overlying skin is removed and examined under a microscope.

It is the best method to confirm whether cancer is present or not.

If cancer is confirmed, the biopsy material is subjected to further testing to see if the cancer cells have hormone receptors (estrogen and progesterone receptors) or if they are producing increased amounts of the HER2 protein (HER2-positive breast cancer). If both estrogen and progesterone receptors are absent and HER2 protein is not being produced excessively, the cancer is labeled as triple-negative breast cancer.

Other tests

Once cancer has been diagnosed, additional testing may be carried out to determine the stage or extent of the disease. This helps to determine the prognosis and the best treatment options available. However complete information about the stage of cancer becomes available only after breast cancer surgery.

Read more about Staging of Breast Cancer

The following additional tests may be performed

Blood tests, such as a complete blood count
Tumor markers such as receptors for estrogen (ER), progesterone (PR) and HER2 along with proliferation factors.
Mammogram of the other breast to look for signs of cancer
Bone scan
Computerized tomography (CT) scan
Positron emission tomography (PET) scan

Stages of Inflammatory Breast Cancer

All inflammatory breast cancers start as stage III as they have already involved the skin.

Inflammatory breast cancer is usually in one of three stages:

Stage III B: Cancer has spread to areas near the breast, such as the skin or chest wall, including the ribs and the chest muscles. It may have spread to lymph nodes within the breast or under the arm.
Stage III C: Cancer has spread to lymph nodes around the collarbone and near the neck. It may have spread to lymph nodes within the breast or under the arm and to areas near the breast.
Stage IV: It means that cancer has spread to distant organs (called distant metastasis), most often the bones, liver, brain, or lungs. It is also called metastatic breast cancer or advanced breast cancer.

Read more about Metastasis or Metastatic Disease

Prognosis

Inflammatory breast cancer is an aggressive type of cancer.

It grows quickly, is more likely to have spread at the time of diagnosis, and is more likely to recur after treatment than other types of breast cancer.

The prognosis of inflammatory breast cancer is poorer as compared to other types of breast cancer.

Treatment

The standard treatment of inflammatory breast cancer is pre-surgical reduction or shrinkage of tumor followed by surgery and finally radiotherapy.

Chemotherapy

It is given to shrink the tumor. Two types of chemo drugs are usually given.

An anthracycline, such as doxorubicin (Adriamycin) or epirubicin (Ellence)
A taxane, such as paclitaxel (Taxol) or docetaxel (Taxotere)

Hormonal therapy

Tamoxifen is given if the cancer is estrogen or progesterone receptor-positive. Most inflammatory breast cancers are however hormone-receptor negative.

Targeted therapy

If the cancer is HER2 positive, targeted therapy is given along with the chemotherapy. This includes the drug trastuzumab (Herceptin) or pertuzumab (Perjeta).

Once, the above therapy is able to shrink cancer and reduce the associated swelling and inflammation, surgical treatment is carried out. If however, cancer does not respond to these treatments, additional chemotherapy or radiation therapy may be given. If cancer still does not respond to this additional treatment, the patient can still have surgery to remove the affected breast tissue and lymph nodes.

Surgery

It includes mastectomy and lymph node dissection. The entire affected breast and the lymph nodes under the arm are removed. Since inflammatory breast cancer is an aggressive type of cancer, breast-conserving surgery (partial mastectomy or lumpectomy) and sentinel lymph node biopsy (removal of only a few lymph nodes) are not recommended.

Radiotherapy

Radiation therapy is usually given after surgery.

Combining these treatments has drastically improved the overall survival of inflammatory breast cancer.

References

Bertucci F, Ueno NT, Finetti P, et al. Gene expression profiles of inflammatory breast cancer: correlation with response to neoadjuvant chemotherapy and metastasis-free survival. Annals of Oncology 2014; 25(2):358-365.
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Hance KW, Anderson WF, Devesa SS, Young HA, Levine PH. Trends in inflammatory breast carcinoma incidence and survival: the surveillance, epidemiology, and end results program at the National Cancer Institute. Journal of the National Cancer Institute 2005; 97(13):966-975.
Chang S, Parker SL, Pham T, Buzdar AU, Hursting SD. Inflammatory breast carcinoma incidence and survival: the surveillance, epidemiology, and end results program of the National Cancer Institute, 1975-1992. Cancer 1998; 82(12):2366-2372.
Dawood S, Cristofanilli M. Inflammatory breast cancer: what progress have we made? Oncology (Williston Park) 2011; 25(3):264-270, 273.