Inflammatory Breast Cancer—Difficult to Detect and Deadly

By Jan Tucker, MBA
Edited by Martin Palmer, MD, Medical Oncologist

With all the familiar information about what we can do to detect breast cancer early, it’s important to keep these facts in mind: There is more than one type of breast cancer. You don’t have to have a lump to have breast cancer. Mammograms or ultrasounds do not detect all breast cancers.
Inflammatory Breast Cancer (IBC), a rare but aggressive form of breast cancer, accounts for one to five percent of all breast cancer cases in the United States. Since IBC tends to grow in nests or sheets rather than as a solid tumor, it can spread through the breast and remain undiagnosed for some time, even if symptoms are present.
This cancer is called “inflammatory” because it often presents with redness, swelling, and warmth in the breast caused by the cancer cells blocking the lymphatic system just below the surface of the skin.
IBC often occurs in younger women, and more often in African Americans than in Whites. It can occur in men, but usually at an older age than in women. According to the National Cancer Institute (NCI), one or more of the following symptoms are typical:
• Swelling of the breast, usually sudden, sometimes a cup size in a few days
• Itching
• Pink, red, or dark colored area (called erythema), sometimes with texture similar to the skin of an orange (called peau d’orange)
• Ridges and thickened areas of the skin
• Nipple retraction
• Nipple discharge, may or may not be bloody
• Breast is warm to the touch
• Breast pain (from a constant ache to stabbing pains)
• Change in color and texture of the areola
• Swollen lymph nodes under the arm, above the collarbone, or in both places
These symptoms often develop very quickly, over a period of weeks or months. The symptoms can also occur in benign breast disorders, and they are often confused with mastitis, which is a breast infection treated with antibiotics. One symptom of mastitis that is not a symptom of IBC is a fever. If you are being treated for mastitis and your symptoms are still present after a week, you should seek a breast specialist who can further diagnose your problem.
Diagnosis
Breast cancer is diagnosed by pathologic examination of biopsies of suspicious abnormalities such as a breast lump, changes described in the previous section, or characteristic abnormalities identified on a mammography. IBC is a clinical diagnosis supported by the biopsy showing invasion of the lymphatics of the overlying skin of the affected breast.
IBC is classified as either stage IIIB or IV breast cancer, IV being the highest (the higher the stage, the poorer the prognosis). Staging reflects the extent of spread of a cancer, helps guide treatment recommendations, and influences the prognosis. A stage IIIB cancer describes one that has spread from where it started to nearby tissue (to the skin of the breast in IBC) and is known as “locally advanced.” A stage IV breast cancer has spread to other organs beyond the breast and local lymph nodes.
Prognosis
Since IBC is more likely to have metastasized (spread to a site distant from the primary cancer as opposed to local spread) than non-IBC cancers by the time it is diagnosed, the five-year survival rate for IBC patients is between 25 and 50 percent. This is significantly lower than the survival rate for patients with other breast cancers. However, many factors influence the outcome of cancer, and it is important to discuss your particular situation with your doctor if you are diagnosed with IBC.
Treatment
Defining the stage of a cancer helps the doctor determine the best treatment plan as well as the patient’s prognosis or chances for recovery. IBC requires immediate, aggressive chemotherapy treatment prior to surgery.
The most common order of treatment, according to the IBC Cancer Research Foundation is neoadjuvant chemotherapy (prior to surgery), surgery, more chemotherapy, and radiation. These treatments have several purposes: to remove the primary cancer to achieve locoregional control (by surgical resection and post-operative radiotherapy), to treat distant metastases where it is known to exist at diagnosis (i.e., stage IV), and to reduce the risk of recurrence (with systemic treatment such as chemotherapy). While about two-thirds of patients with all types of breast cancer express estrogen receptors (ER) and/or progesterone receptors (PR), making them more likely to respond to blocking these receptors by hormonal treatment such as Tamoxifen, most patients with IBC do not express these receptors on the surface of their cancer cells and thus generally do not benefit from hormonal treatment.
Patients can receive supportive care to help manage the symptoms related to the cancer and the side effects of its treatment. For example, the skin overlying the affected breast can break down because of cancerous erosion through the surface, leaving the patient open to complicating bacterial infection that requires antibiotics as well as careful attention to wound care.
The standard of care for IBC generally involves multi-modality treatment with combined use of chemotherapy, surgery, and radiotherapy, and there are a variety of acceptable choices, particularly for the specific chemotherapy drugs used. Thus the specifics of treatment can vary by doctor, by the institution, by disease stage at presentation, and can also be influenced by where the patient lives (affecting treatment resources locally available), and by their individual medical history (influencing their ability to tolerate side effects of treatment).
Clinical Trials
The National Cancer Institute (NCI), part of the National Institutes of Health of the U.S. Department of Health and Human Services and the Federal Government’s principal agency for cancer research, is sponsoring clinical trials designed to find new treatments and better ways to use current treatments. Speak with your doctor if you are interested in participating in a clinical trial. Information is available by calling 1-800-4CANCER (422-6237) or by visiting http://www.cancer.gov/publications or http://www.cancer.gov/clinicaltrials.
IBC Patients Describe Their Symptoms
IBC is difficult to diagnose yet early detection can be critical to the patient’s chances for survival. Because of this, IBC Research has posted a list of patients’ own words describing their symptoms prior to being diagnosed with IBC. Please see http://www.ibcresearch.org/symptoms/ibc-patients-write for the complete list.
Some brief descriptive words and phrases common in this list are redness; swollen lymph nodes; a lump (often large and growing); change in the size, appearance, or feeling in one breast; hardening of a breast; appearance of a rash, starburst, reddish, bruise-like, or other mark; uncomfortably sensitive breast; constant, intense itching of the breast; a discomfort under the arm. One IBC patient’s doctor told her that often one breast gets larger as you get older.
Several patients described how they rationalized there wasn’t a problem, even though they experienced changes. “I thought I was having a reaction to new [detergent or bra],” or “I was not concerned about this because of [other breast problems I had with similar symptoms],” and “I thought cancerous lumps weren’t painful.”
There are many ways to rationalize or overlook changes we are experiencing. The risk of breast cancer is higher than we may realize: One out of eight women will be diagnosed with breast cancer. According to statistics, one to five women out of every 100 diagnosed will have IBC. The best defense is your awareness of this silent, devastating disease. Do not ignore or rationalize your symptoms. Have them checked immediately.
Please visit the following Web sites, which were also used to research this article: http://www.cancer.gov and http://www.ibcresearch.org.
Dr. Martin Palmer is in oncology practice with Ashwin Kashyap, MD; and Robert Joseph, MD; located at 1240 Westlake Blvd, Suite 117 in Westlake Village. Dr. Palmer may be reached at 805-496-0592.