IBC: A different type of breast cancer
Inflammatory breast cancer (IBC) is a relatively rare type of breast cancer that grows in the lymph vessels of the skin of the breast. Because it usually doesn’t form an easy-to-find lump and because it tends to spread rapidly, IBC is the most deadly form of breast cancer. No matter how quickly IBC is diagnosed, it is considered at least Stage IIIB because the cancer is already in the lymphatic system. According to Dr. Beth Overmoyer in a presentation at the 2015 San Antonio Breast Symposium, in about 35% of IBC patients, the cancer has already spread to other organs at diagnosis, which makes it a Stage IV cancer for those patients.
The median age of IBC patients is about 57, compared to over 62 years old for other breast cancers, but much younger women often get IBC. Statistics for IBC vary, but in North America, IBC accounts for about 1% to 5% of all breast cancers. The IBC rate for women of African descent may be as high as 10%.
For a long time, doctors considered IBC to be regular breast cancer cells that were more dangerous because they were in the lymph system. Recent research, however, is finding that inflammatory breast cancer cells tend to be different in some ways from other forms of breast cancer. In a study at New York University's Cancer Institute at Langone Medical Center released June 14, 2009 in Nature, scientists "have identified a key gene-eIF4G1-that is overexpressed in the majority of cases of IBC, allowing cells to form highly mobile clusters that are responsible for the rapid metastasis that makes IBC such an effective killer."
Compared to non-IBC breast cancer, IBC tumors are more often ER/PR negative and Her-2 positive. About 90% of the time patients have positive lymph nodes.
Different Symptoms: What to look forInflammatory breast cancer’s symptoms are not the ones women are trained to look for. Although many IBC patients have a lump, the majority do not.** The characteristic redness, swelling and skin dimpling most associated with IBC are caused by cancer cells in the lymph vessels blocking the normal flow of lymph fluid.** The symptoms vary quite a bit from patient to patient. However, most patients will have several of these symptoms.
- Rapid change in the appearance of one breast, within less than six months
- Thickness, heaviness or visible enlargement of one breast
- Discoloration, giving the breast a red, purple, pink or bruised appearance covering a large portion of the breast
- Unusual warmth of the affected breast
- Dimpling or ridges similar to the peel of an orange
- Itching, often deep inside the breast
- Tenderness, pain or aching
- Enlarged lymph nodes under the arm or near the collarbone
- Recent flattening or turning inward of the nipple or other changes in texture or color in the nipple area
Women who have any of these symptoms for more than a week should see their doctor. However, just one symptom should not be a cause for panic. In fact, all of these symptoms are also symptoms of other breast problems that are not dangerous.** A Different Diagnostic Process with Complicating Factors**
Inflammatory breast cancer presents several challenges for diagnosis. First, the symptoms overlap with infections like mastitis. Second, most IBC patients do not have a lump that shows up on a mammogram. Third, most primary care doctors rarely see a case of IBC, so they may not realize that a patient has it.
The facts that patients may be much younger than typical breast cancer patients and that the presentation symptoms vary widely depending on which lymph vessels are blocked make IBC complicated to diagnose.
Because IBC symptoms typically look like any of several types of infections, most doctors will start treatment with an antibiotic. This is a sensible measure because infections are common and IBC is rare. The response (or lack of one) to the antibiotic is useful information in making the diagnosis.
If the condition goes away with medicine, then the problem isn’t cancer. The Inflammatory Breast Cancer Research Foundation** recommends that if the antibiotic doesn’t work within a week that a biopsy be performed.**
Another part of diagnosis usually includes some imaging tests. Sometimes IBC is referred to as a cancer that can’t be found on a mammogram. However, some women with IBC do have an underlying mass that will show up on a mammogram. About 70% of women with IBC have changes in their mammograms in skin thickness or breast density as well as other abnormalities. So it is important for patients to ask for their new images to be compared to previous ones. Ultrasounds and breast MRI’s may also find changes that indicate the possibility of IBC. They can guide the doctor to the best area for a biopsy.
Ultimately the diagnosis of IBC rests on a biopsy that shows cancer cells in the skin of the breast. If a patient suspects that she has IBC, she should contact her primary care doctor who can start the diagnostic process. If initial treatment doesn’t resolve her symptoms, she should ask for a referral to a breast surgeon who can do a biopsy.Patients have an important role in the diagnostic process. Doctors are sometimes reluctant to suspect IBC because it is rare. Doctors who have seen IBC cases before may be thrown off track by a patient whose symptoms that don’t fit their previous experience. Unfortunately, some women with IBC still hear, “You are too young to have breast cancer,” or “The mammogram is clear, nothing to worry about,” from their doctors. It’s important to be persistent in getting an accurate diagnosis when IBC is a possibility.** The Importance of Trimodal Treatment**
In 2008 the National Comprehensive Cancer Network (NCCN) recognized that IBC is a distinct form of cancer that needs its own treatment guidelines. Many forms of breast cancer can be treated with just one or two types of treatment, but IBC patients do best with trimodality--a combination of three types of treatment
The current best practice for treating IBC starts with chemotherapy. Because doctors know that the cancer is already in the lymphatic system, they worry that those cancer cells have spread to vital organs. Chemotherapy is a systemic treatment that can kill those wandering cells right away.
After chemotherapy, a mastectomy with lymph node removal is the preferred surgery. Unfortunately, since there isn’t usually a lump and since the cancer is in the skin, a lumpectomy is not possible.
Depending on the results of the pathology report, more chemo may be done after surgery. If the pathology looks good, the patient proceeds to radiation treatments.
The next steps depend on the characteristics of the cancer. If the patient’s tumor cells were Estrogen Receptor positive, then she will receive hormone therapy. If they were HER2 positive, she will get** Herceptin or another targeted therapy.**** Better treatments lead to improved outcomes**
Because IBC has a high recurrence rate within the first three years, doctors follow their IBC patients very carefully with frequent physical exams and blood work. If any symptoms of possible metastasis appear, doctors will order further imaging tests or biopsies to check to see if the cancer has spread.
At one time IBC was considered a certain death sentence. However, treating it with chemo first, followed by surgery and radiation has dramatically increased survival rates. An individual woman's prognosis depends on the characteristics of her tumor and her stage at diagnosis, so each woman needs to discuss her situation with her own doctor.
Being diagnosed with IBC is a frightening experience. It’s important for new patients to realize that any statistics they read do not predict what will happen to them and to live in the hope that they will survive to accomplish their goals in life. At one time IBC was often excluded from breast cancer research because of its rarity. Now researchers are realizing that understanding the very factors that make IBC so deadly will help them unlock the secrets of other types of breast cancer. There is definitely hope for IBC patients.
For Further Reading
Merajver, S. Diagnostic challenges, epidemiology, and updates in the search for IBC signaling determinants. Lecture. San Antonio Breast Cancer Symposium, Dec. 10, 2013. Retrieved from http://sabcs.org/pastsymposia/index.asp
NIH Fact Sheet: Inflammatory Breast Cancer Retrieved from http://www.cancer.gov/cancertopics/factsheet/Sites-Types/IBC
IBC Research Foundation. Symptoms of IBC. Accessed Feb. 23, 2014. http://www.ibcresearch.org/symptoms/