Rare Breast Cancer Poses New Set of Challenges

FRIDAY Oct. 5, 2012 -- No doubt the ubiquitous pink ribbons, along with walks and races and the designation of October as National Breast Cancer Awareness Month, have focused the public eye on breast cancer and helped fund research for a cure.

But chances are good that most people still haven't heard of a particularly aggressive type of the disease known as inflammatory breast cancer.

Though rare, involving 1 to 5 percent of breast cancer cases, according to the U.S. National Cancer Institute, inflammatory breast cancer poses significant hurdles. Mammograms often aren't effective in detecting this type of cancer because there may be no discernible lump. Also, standard breast cancer treatments aren't always effective for inflammatory breast cancer.

"This disease develops quickly over a few weeks," explained Dr. Massimo Cristofanilli, a professor of medicine and director of translational medicine at Fox Chase Cancer Center in Philadelphia. "This cancer has the capacity to spread to the lymph nodes and distant sites quickly."

The exact cause of inflammatory breast cancer is unknown, he said. According to the cancer institute, it usually develops in the cells that line the milk ducts and then spreads to other areas.

The primary symptoms are redness and noticeable swelling. Cristofanilli said that skin on the breast can resemble that of an orange peel. The disease is named inflammatory breast cancer because the breast looks inflamed. The swelling occurs because the lymph vessels in the skin become blocked and fluid doesn't drain properly.

"Most women will present rapidly with an engorged breast. Their bra won't fit anymore," said Dr. Naoto Ueno, executive director of the Morgan Welch Inflammatory Breast Cancer Research Program and Clinic, and a professor in the department of breast medical oncology at the University of Texas M.D. Anderson Cancer Center in Houston.

"At this point, women often go to a primary care physician," Ueno said. "They will usually diagnose mastitis, an infection of the breast. If you've had one to two weeks of antibiotics, and you're not getting better, it could be inflammatory breast cancer, and you should have a biopsy." A biopsy of breast tissue can confirm a diagnosis.

Cristofanilli said that "women need to remember that mammogram is not helpful for inflammatory breast cancer, so there's usually no early diagnosis." That factor combined with the aggressive nature of the disease means that the cancer often has metastasized, or spread to other parts of the body, by the time a woman sees a cancer specialist.

"Making a diagnosis and getting a biopsy quickly are critical," he said.

The average survival for women diagnosed with inflammatory breast cancer is about three years, Cristofanilli said. For someone whose cancer has spread beyond breast tissue but not to distant sites, considered a stage 3 cancer, the average survival time extends to about five years with the latest multi-disciplinary care.

Ueno confirmed that many of the currently available treatments don't do a great job with inflammatory breast cancer. "With stage 4 inflammatory breast cancer, the five-year survival is only 30 to 40 percent," even with the best available treatment, he said. Stage 4 cancer has spread to distant sites, such as the lungs or brain.

Both experts said it's important to seek care at a center with a lot of experience in treating inflammatory breast cancer. For a woman who doesn't live close to such a center, Ueno suggested that she visit for an initial diagnosis and treatment plan and then arrange for the center's specialists to work with her local cancer experts.

The first treatment for inflammatory breast cancer is chemotherapy, and possibly hormone therapy, according to Ueno and Cristofanilli. The goal is to eliminate any signs of the disease before doing a mastectomy. Because inflammatory breast cancer usually involves the whole breast, and doesn't generally cause a lump, mastectomy is the only surgical option. Ueno said that surgery is usually followed by aggressive radiation.

If inflammatory breast cancer recurs after treatment, both experts suggested checking into clinical trials because researchers are constantly trying to find better ways to treat this type of cancer.

As with any cancer, the earlier it's detected, the better.

"If you have any redness or swelling, see a breast specialist right away," Cristofanilli urged. "Don't delay."

More information

The American Cancer Society has more on inflammatory breast cancer.

A companion article details a survivor's story.

Posted: October 2012


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