Rare, Aggressive Breast Cancer Hard to Detect and Diagnose

Generally, most of the news about breast cancer has been encouraging: Thanks to advances in diagnostic techniques over the past 25 years, most breast cancers are detected in early stages. That's when treatment is most effective, and today's newer treatments have added to survival. Although more than 200,000 cases of breast cancer are diagnosed every year in the United States, the disease now causes death in only about 41,000 cases annually.

At the same time, a little-known, aggressive type of breast cancer - called inflammatory breast cancer, or IBC - remains difficult to diagnose until it's well-advanced and difficult to treat. The National Cancer Institute (NCI) describes IBC as a disease that occurs when cancer cells block the lymph vessels in the skin of the breast.

Fortunately, IBC is rare, accounting for only 2% to 5% of breast cancers, says Yee Chung Cheng, MD, an Assistant Professor of Medicine (Neoplastic Diseases and Related Disorders) at the Medical College of Wisconsin, and Chief of Hematology/Oncology at the Zablocki Veterans Affairs Medical Center. It's a confounding disease, however: Unlike other types of breast cancer, IBC commonly has no palpable lump, and it rarely shows up on a mammogram as a dominant mass.

Symptoms of IBC
"The typical symptoms are a red, swollen breast that may feel unusually warm," Dr. Cheng says, adding that it's easy to confuse such symptoms with an irritation from an insect bite, an infection or mastitis, which some women feel shortly after giving birth.

Other IBC symptoms can include changes in the breast skin that resemble the texture of an orange (peau d'orange), persistent itching, and dimpling or pleating of the breast. Unlike some infections it mimics, however, IBC is not accompanied by a fever, nor can antibiotics relieve it.

"Unfortunately, most patients don't suspect cancer until it's in the late stages," Dr. Cheng says. "No lump can be felt, and even a mammogram might not indicate a tumor. Occasionally with this condition, a mammogram might show a mass, microcalcification or some thickening of the skin which raise the suspicion of malignancy."

Who's at Risk
As with more common types of breast cancer, African-American women are at greater risk for IBC than Caucasians, Dr. Cheng says. It can occur in men, although - like breast cancer generally - it's overwhelmingly a women's disease. The American Cancer Society estimates that breast cancer is about 100 times more common among women than men.

IBC can affect people of all ages, and it seems more common in young patients. In May 2006, a Seattle television station, KOMO, reported on the death of a 16-year-old girl from IBC.

Because IBC is so unusual, the KOMO report found, some cancer help lines had no information about it. In fact, many physicians never see a case during their practice. Since there is no known remedy or regimen to prevent IBC, it is important that patients are assertive with health care providers who are unaware of the symptoms of IBC - even doctors might need some information on the topic in order to follow up with tests.

Diagnosing IBC
"The most definitive way to confirm that the patient has breast cancer is with a biopsy, but the diagnosis of IBC is based on biopsy and the presence of appropriate clinical findings on the breast," Dr. Cheng says.

Because IBC affects the lymph channels in the skin of the breast, it can grow rapidly and spread throughout the body. That, combined with its ambiguous symptoms, is why IBC is often advanced when it's finally diagnosed - a Stage 3 already or even Stage 4 cancer if there is other distant involvement. The four stages of cancer are used to determine how far a cancer has spread and which treatment protocols to follow.

Treating IBC Aggressively
"With more common types of breast cancer," Dr. Cheng says, "the treatment usually begins with surgery, either a lumpectomy or mastectomy, along with a node-mapping procedure, in which a few lymph nodes are removed and examined to determine whether the cancer has spread to the lymph nodes in the armpit." A lumpectomy is often followed by radiation to prevent a recurrence. In some cases, patients also receive a course of chemotherapy or hormone therapy or both after surgery.

"With IBC, the treatment is just the reverse," he continues. "With IBC, we don't advocate just a lumpectomy or a sentinel lymph node biopsy. Since we know the lymph system is affected and the disease is more advanced, we take care of it first, with extensive chemotherapy. If patients respond to the chemotherapy either completely or partially, we do a mastectomy and remove the lymph nodes in the armpit to prevent a relapse. Then we follow up with radiation. We generally follow the National Cancer Institute guidelines." In some cases, patients are prescribed additional systemic treatment with hormonal therapy, or trastuzumab if indicated. Trastuzumaub (commonly known as Herceptin) is a monoclonal antibody, a class of drugs that works by interfering with one of the ways in which breast cancer cells divide and grow.

Using the standard 5-year disease-free survival rates, Dr. Cheng noted that the importance of combined multidiscipline treatment for IBC patients produces better results. He cited these survival-rate findings:

• 40% for IBC patients undergoing chemotherapy, followed by surgery, then radiation
• 24% for IBC patients undergoing surgery, followed by radiation
• 6% for IBC patients who had only surgery or only radiation

"It's a very bad type of cancer," Dr. Cheng agrees. Nevertheless, he says: "Don't get scared. It's not a common disease." Meantime, his advice: "Follow the American Cancer Society's recommendations for breast health. Have a clinical breast exam starting at age 20, and every three years afterward. Do breast self-examinations regularly, and at 40, start having mammograms as recommended.

"Pay attention to routine health care," says Dr. Cheng. "Follow a sensible lifestyle, and be aware of cancer warning signs." And remember the typical - though sometimes vague - symptoms of IBC: a red, swollen breast that may feel unusually warm; changes in the breast skin that resemble the texture of an orange, persistent itching, and dimpling or pleating of the breast. If you experience these symptoms, see your doctor immediately and explain your concerns.

Barbara Abel
HealthLink Contributing Writer

MCW Health News presents up-to-date information on patient care and medical research by the physicians of the Medical College of Wisconsin.