Posted on February 5th, 2009 by
By Kari Bohlke, ScD
A short quiz: You notice redness, warmth, and swelling in one of your breasts, but you don’t feel a lump and your most recent mammogram was normal. Should you be concerned about breast cancer?
Answer: Possibly. And you should certainly discuss your symptoms with your healthcare provider without delay. An aggressive type of breast cancer known as inflammatory breast cancer (IBC) can produce these and other symptoms. Inflammatory breast cancer often spreads in sheets, rather than forming a tumor that feels like a lump, and is frequently missed by mammography prior to the development of symptoms.
Women who have had mastitis (a breast infection that most commonly occurs in breastfeeding women) will notice that the symptoms just mentioned are similar to those of mastitis. Unlike mastitis, however, IBC is generally not accompanied by a fever and will not respond to treatment with antibiotics. If you’re being treated for mastitis but notice that your symptoms are not improving, you may wish to talk with your doctor about a breast biopsy or a referral to a breast specialist.
What Is Inflammatory Breast Cancer?
IBC is an aggressive type of breast cancer with symptoms that differ from those of other types of breast cancer (see sidebar). The redness, warmth, and swelling that often accompany IBC are caused by the blockage of lymph vessels by cancer cells.1 At the time of diagnosis, most women with IBC will have lymph node metastases, and roughly one-third will have distant metastases.2
“It’s not a new disease,” explains Dr. Massimo Cristofanilli, co-director of the Inflammatory Breast Cancer Clinic at the University of Texas M. D. Anderson Cancer Center, “but it’s a disease that has not been explored the way it should have been over the years.”
The diagnosis of IBC is based on the rapid development of characteristic symptoms, coupled with the presence of cancer cells on a breast biopsy.2 The most common type of cancer found in women with IBC is invasive ductal carcinoma, which is also the most common type found in women with non-inflammatory breast cancer. In the case of IBC, however, the cancer cells grow and spread very differently. The reason for this different growth pattern is still unknown but is an active area of research.
“To design better treatment and to develop ways to prevent inflammatory breast cancer, we need to understand why this disease is so different,” says Dr. Cristofanilli.
How Common Is Inflammatory Breast Cancer?
Inflammatory breast cancer is less common than non-inflammatory breast cancer, but its poor prognosis highlights the importance of raising awareness about it. IBC accounts for an estimated 2 percent of breast cancer diagnoses in the United States but 7 percent of breast cancer deaths.3 Furthermore, from the late 1980s to the late 1990s, the incidence of IBC increased by roughly 25 percent.3
While these numbers—which are generated from cancer registry data—provide a glimpse into the frequency of IBC, Dr. Cristofanilli points out that they are likely to underestimate the true frequency of IBC. “Many times inflammatory breast cancer is misdiagnosed,” he explains. IBC is likely to become a more prevalent health issue for women in the future as other types of breast cancer continue to decline and recognition of IBC increases.
Who Is at Risk of Inflammatory Breast Cancer?
Risk factors for IBC are poorly understood, and currently it is not possible to estimate a woman’s risk of this type of cancer. IBC tends to occur at a younger age than other types of breast cancer, however, prompting interest in the role of genetic predisposition and early-life exposures.3 Rates of IBC increase rapidly up to age 50 and then stabilize. IBC is also more common in African-American women than in White women.
Women who develop symptoms of IBC should seek immediate care and should be prepared to take an active role in the investigation of their symptoms. Dr. Cristofanilli notes that he’s known several patients who were diagnosed with inflammatory breast cancer only after they questioned their physician’s diagnosis of mastitis and asked specifically about the possibility of IBC.
How Is Inflammatory Breast Cancer Treated?
Treatment of IBC generally begins with chemotherapy,4 a systemic (whole-body) treatment. The objective of chemotherapy is both to eliminate areas of cancer that have already spread beyond the breast and to reduce the amount of cancer in the breast prior to locoregional therapy (therapy delivered to the breast and the surrounding tissues).
Depending on the nature of the cancer and its response to initial chemotherapy, locoregional therapy consists of radiation therapy alone or surgery to remove the breast and the nearby lymph nodes coupled with radiation therapy. Patients often then receive additional systemic therapy, which may include additional chemotherapy, hormonal therapy, targeted therapy, or a combination of these approaches.1
What Is the Prognosis of Inflammatory Breast Cancer?
Survival with IBC is worse than with other types of breast cancer, with an estimated 25 to 50 percent of women surviving for at least five years.1 While these numbers are sobering, they represent an important improvement over the past. The addition of chemotherapy to locoregional therapy with surgery and/or radiation has allowed some women with IBC to become long-term survivors.5 Nevertheless, further improvements in treatment are clearly needed.
What Are Promising Areas of Research?
Because the prognosis of IBC remains worse than for other types of breast cancer, identifying improved approaches to the treatment of IBC is an important priority. Treatments currently being evaluated in clinical trials include new targeted agents such as Tykerb® (lapatinib).6 Tykerb targets two proteins—EGFR and HER2—that are abnormally expressed in many (but not all) cases of IBC. Inhibiting these proteins can slow or stop cancer growth.
In addition to aberrant expression of EGFR and HER2, several other factors are likely to contribute to the development and the growth of IBC cells. Research is under way to better understand these factors, with the goal of identifying weaknesses of IBC cells that could be targeted by new therapeutic approaches.
For many women diagnosed with IBC, the shock of a breast cancer diagnosis is compounded by the diagnosis of a type of breast cancer that they’ve never heard of before. Learning about this cancer in advance can help you recognize its symptoms and seek prompt treatment.
The opening of the world’s first clinic devoted solely to the study and the treatment of IBC (see sidebar) offers additional hope. The clinic promises to offer state-of-the-art care while building our understanding of how this cancer works and how it can be stopped.
Symptoms of Inflammatory Breast Cancer1
These symptoms can also be caused by conditions other than cancer, and it is important for you to discuss them with your healthcare provider.
3. 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):966-75.
4. Cristofanilli M, Buzdar AU, Hortobágyi GN. Update on the management of inflammatory breast cancer. The Oncologist. 2003;8(2):141-48.
5. Giordano SH, Hortobágyi GN. Inflammatory breast cancer: Clinical progress and the main problems that must be addressed. Breast Cancer Research. 2003;5(6):284-88.
6. Cristofanilli M, Boussen H, Baselga J, et al. A Phase II combination study of lapatinib and paclitaxel as a neoadjuvant therapy in patients with newly diagnosed inflammatory breast cancer. Paper presented at: 2006 Annual San Antonio Breast Cancer Symposium; oral presentation December 14, 2006; San Antonio, Texas. Abstract 1.
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