Inflammatory breast cancer

Overview

Inflammatory breast cancer is a rare but very aggressive type of breast cancer in which the cancer cells block the lymph vessels in the skin of the breast. This type of breast cancer is called “inflammatory” because the breast often looks swollen and red, or “inflamed.” IBC accounts for 1 to 5 percent of all breast cancer cases in the United States (1). It tends to be diagnosed in younger women compared to non-IBC breast cancer. It occurs more frequently and at a younger age in African Americans than in Whites. Like other types of breast cancer, IBC can occur in men, but usually at an older age than in women. Some studies have shown an association between family history of breast cancer and IBC, but more studies are needed to draw firm conclusions (2).

Symptoms

Symptoms of IBC may include redness, swelling, and warmth in the breast, often without a distinct lump in the breast. The redness and warmth are caused by cancer cells blocking the lymph vessels in the skin. The skin of the breast may also appear pink, reddish purple, or bruised. The skin may also have ridges or appear pitted, like the skin of an orange (called peau d'orange), which is caused by a buildup of fluid and edema (swelling) in the breast. Other symptoms include heaviness, burning, aching, increase in breast size, tenderness, or a nipple that is inverted (facing inward) (3). These symptoms usually develop quickly—over a period of weeks or months. Swollen lymph nodes may also be present under the arm, above the collarbone, or in both places. However, it is important to note that these symptoms may also be signs of other conditions such as infection, injury, or other types of cancer (1).

Causes

The cause of breast cancer isn't known, but its high incidence in women implicates estrogen. Certain predisposing factors are clear; women at high risk include those who have a family history of breast cancer, particularly first-degree relatives (mother, sister, and maternal aunt). Other women at high risk include those who: *have long menstrual cycles or began menses early (before age 12) or menopause late (after age 55) *have taken hormonal contraceptives *used hormone replacement therapy for more than 5 years *who took diethylstilbestrol to prevent miscarriage *have never been pregnant *were first pregnant after age 30 *have had unilateral breast cancer *have had ovarian cancer — particularly at a young age *were exposed to low-level ionizing radiation. Recently, scientists have discovered the BRCA1 and BRCA2 genes. Mutations in these genes are thought to be responsible for less than 10% of breast cancers. However, these discoveries have made genetic predisposition testing an option for women at high risk for breast cancer. Women at lower risk include those who: *were pregnant before age 20 *have had multiple pregnancies *are Native American or Asian. Most breast cancer deaths occur in women age 50 and older (84% of cases), and 77% of new breast cancer cases occur in this age-group. However, it may develop any time after puberty. It occurs in men, but rarely; male cases of breast cancer account for less than 1% of all cases. The 5-year survival rate for localized breast cancer has improved because of earlier diagnosis and the variety of treatments now available. According to the most recent data, mortality rates continue to decline in White women and, for the first time, are also declining in younger Black women. Lymph node involvement is the most valuable prognostic predictor. With adjuvant therapy, 70% to 75% of women with negative nodes will survive 10 years or more compared with 20% to 25% of women with positive nodes.

Diagnosis

Diagnosis of IBC is based primarily on the results of a doctor's clinical examination (1). Biopsy, mammogram, and breast ultrasound are used to confirm the diagnosis. IBC is classified as either stage IIIB or stage IV breast cancer (2). Stage IIIB breast cancers are locally advanced; stage IV breast cancer is cancer that has spread to other organs. IBC tends to grow rapidly, and the physical appearance of the breast of patients with IBC is different from that of patients with other stage III breast cancers. IBC is an especially aggressive, locally advanced breast cancer. Cancer staging describes the extent or severity of an individual's cancer. (More information on staging is available in the National Cancer Institute (NCI) fact sheet Staging: Questions and Answers at http://www.cancer.gov/cancertopics/factsheet/Detection/staging on the Internet.) Knowing a cancer's stage helps the doctor develop a treatment plan and estimate prognosis (the likely outcome or course of the disease; the chance of recovery or recurrence). Home medical testing related to Inflammatory breast cancer: * Colon & Rectal Cancer: Home Testing o Home Colorectal Cancer Tests o Home Fecal Occult Bleeding Tests * Breast Cancer: Related Home Tests: o Home Breast Cancer Tests o Home Breast Lump Detection

Prognosis

Prognosis describes the likely course and outcome of a disease—that is, the chance that a patient will recover or have a recurrence. IBC is more likely to have metastasized (spread to other areas of the body) at the time of diagnosis than non-IBC cases (3). As a result, the 5-year survival rate for patients with IBC is between 25 and 50 percent, which is significantly lower than the survival rate for patients with non-IBC breast cancer. It is important to keep in mind, however, that these statistics are averages based on large numbers of patients. Statistics cannot be used to predict what will happen to a particular patient because each person's situation is unique. Patients are encouraged to talk to their doctors about their prognosis given their particular situation.

Treatment

Treatment consisting of chemotherapy, targeted therapy, surgery, radiation therapy, and hormonal therapy is used to treat IBC. Patients may also receive supportive care to help manage the side effects of the cancer and its treatment. Chemotherapy (anticancer drugs) is generally the first treatment for patients with IBC, and is called neoadjuvant therapy. Chemotherapy is systemic treatment, which means that it affects cells throughout the body. The purpose of chemotherapy is to control or kill cancer cells, including those that may have spread to other parts of the body. After chemotherapy, patients with IBC may undergo surgery and radiation therapy to the chest wall. Both radiation and surgery are local treatments that affect only cells in the tumor and the immediately surrounding area. The purpose of surgery is to remove the tumor from the body, while the purpose of radiation therapy is to destroy remaining cancer cells. Surgery to remove the breast (or as much of the breast tissue as possible) is called a mastectomy. Lymph node dissection (removal of the lymph nodes in the underarm area for examination under a microscope) is also done during this surgery. After initial systemic and local treatment, patients with IBC may receive additional systemic treatments to reduce the risk of recurrence (cancer coming back). Such treatments may include additional chemotherapy, hormonal therapy (treatment that interferes with the effects of the female hormone estrogen, which can promote the growth of breast cancer cells), targeted therapy (such as trastuzumab, also known as Herceptin®), or all three. Trastuzumab is administered to patients whose tumors overexpress the HER–2 tumor protein. More information about Herceptin and the HER–2 protein is available in the NCI fact sheet Herceptin® (Trastuzumab): Questions and Answers, which can be found at http://www.cancer.gov/cancertopics/factsheet/therapy/herceptin on the Internet. Supportive care is treatment given to improve the quality of life of patients who have a serious or life-threatening disease, such as cancer. It prevents or treats as early as possible the symptoms of the disease, side effects caused by treatment of the disease, and psychological, social, and spiritual problems related to the disease or its treatment. For example, compression garments may be used to treat lymphedema (swelling caused by excess fluid buildup) resulting from radiation therapy or the removal of lymph nodes. Additionally, meeting with a social worker, counselor, or member of the clergy can be helpful to those who want to talk about their feelings or discuss their concerns. A social worker can often suggest resources for help with recovery, emotional support, financial aid, transportation, or home care.