Inflammatory breast cancer: a stealthy, aggressive foe

It usually doesn't cause lumps and its symptoms mimic breast infections, but it accounts for about 5 percent of all breast-cancer cases and a higher percentage of breast-cancer deaths.

By Nick Perry
Seattle Times staff reporter

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At first, Nancy Key figured the itchy spot on her breast was a spider bite.

But after a few weeks, the spot became red, hot and unbearably itchy. Her breast swelled a little. The surrounding skin turned thick and dimpled, like an orange peel.

Two days after contacting her doctor, Key was sitting in a surgeon's office, the results of a biopsy back. The surgeon told Key she had a particularly deadly form of breast cancer: inflammatory breast cancer, a disease that usually doesn't cause breast lumps.

"I was shocked, I had never heard of it," Key said. "I had done everything right: a breast self-exam every month, and mammograms every year.

"I didn't know there was a type of breast cancer without a lump," Key said. "I kept thinking it wasn't real. I feel fine, I don't have a lump, how can I have the worst breast cancer in the world?"

Key's shock is echoed by many patients who have never heard of the disease before diagnosis. Even doctors often mistake the symptoms for mastitis, a breast infection, or a side-effect of menopause.

Yet studies indicate inflammatory breast cancer accounts for about 5 percent of all breast-cancer cases and a disproportionately high ratio of breast-cancer deaths.

Key's symptoms are typical. Other signs can include a retracted nipple and purple or bruised-looking skin. In most cases there is no tumor, which explains why the disease can be missed on mammograms and self-exams. Inflammatory breast cancer tends to take hold more quickly and strike younger women than more common breast cancer.

Inflammatory breast cancer Occurs when cancer cells attack the lymphatic system. It accounts for about one in 20 breast-cancer cases but a higher ratio of deaths. Symptoms can include breast changes such as: redness, itchiness, swelling, warmth, pain, firmness and the breast skin turning thick and pitted in an effect called "peau d'orange." Nipples sometimes become retracted. Sometimes a red, pink or dark bruiselike spot appears. Tumors often cannot be felt, nor detected by a mammogram or an ultrasound. See your doctor immediately if you notice breast changes. Doctors typically test for the disease by taking a biopsy of breast tissue, either from surface tissue or from deeper in the breast. The cancer grows and spreads rapidly, often to other parts of the body, resulting in high death rates. Support groups recommend women insist on a biopsy if they believe they have some of the symptoms. Treatment typically begins with chemotherapy then progresses to mastectomy and radiation therapy. Sometimes patients take medication to block estrogen, which can fuel the cancer. Even patients who respond well to treatment are never considered cured.

Swelling and itching is caused when the cancer invades the lymphatic system surrounding the breast, leading to a build-up of lymphatic fluid.

Even survivors like Key, who was 47 when diagnosed in 1998, are never told they are cured or that the cancer is in remission. The nature of the cancer means it has already moved within the lymphatic system by the time it is diagnosed, so patients will receive a lifetime of treatment, in hopes the cancer doesn't flare up again.

The disease is so aggressive that more than half of patients die within five years — compared with fewer than 14 percent of all breast-cancer patients. In all, about 40,000 U.S. women die annually from breast cancer, making it the leading cause of death for women aged 20 to 59. About 10,000 women contract inflammatory breast cancer each year.

Fast spreading

Despite being recognized as a separate condition in the 1920s, inflammatory breast cancer remained little studied until the late 1990s. The medical community paid most attention to early cancer intervention, which provides the best results. Early intervention is almost impossible with inflammatory breast cancer — because the disease spreads quickly.

Even now, experts differ in their definition of the disease, leaving statistics open to interpretation. Some classify breast cancer as being inflammatory only when a certain percentage of the breast has taken on the orange-peel appearance, termed "peau d'orange." Others apply the inflammatory label more liberally.

Owen Johnson, who helps run a nationwide advocacy group from his Bainbridge Island home, experienced firsthand the lack of knowledge and confusion about the disease.

Information National Cancer Institute: www.cancer.gov Inflammatory Breast Cancer Research Foundation: www.ibcresearch.org Inflammatory Breast Cancer Support: www.ibcsupport.org Susan Love M.D. Web site for women: www.susanlovemd.org

Johnson's late wife, Marilyn, woke one spring morning in 1996 noticing her left breast was larger than her right breast. Her husband recalls her asking "Owen, what's just happened?"

The couple consulted their obstetrician and gynecologist. He took a mammogram, which did not reveal any lumps, and he concluded the symptoms were related to menopause. When Marilyn Johnson noticed a light pink spot about the size of a half-dollar some months later, the same specialist initially thought it was mastitis and prescribed antibiotics.

A breast surgeon finally diagnosed Marilyn Johnson's condition in January 1997. Marilyn died 15 months later, 15 days after the couple's 20th wedding anniversary.

Left frustrated and wanting to help others, Owen Johnson began asking questions about the disease and then launched the nonprofit Inflammatory Breast Cancer Research Foundation about a year after his wife's death.

He regularly sends newsletters and e-mails to more than 1,200 survivors and their families. His group has teamed with other disease-advocacy groups with the aim of funding a clinical database that would store tissue samples and medical records, which could be used for research.

"Things are opposite"

One group of researchers that has been studying the disease intently is based out of the University of Michigan.

"Up until about six or seven years ago, not very much was known about it," said Kenneth van Golen, a researcher at the University of Michigan. "Basically, we found a couple of genetic differences."

One of the genes that features prominently in inflammatory breast cancer is called RhoC GTPase. Van Golen believes that gene may help the cancer move and spread out. RhoC GTPase is prominent in other aggressive cancers such as pancreatic cancer.

Meanwhile, a different gene that likely encourages the formation of cancerous lumps in more typical breast cancer is less prominent in the inflammatory disease, van Golen said.

"It's a very strange type of cancer where things are opposite" to common breast cancer, he said.

Treatment also tends to be a little topsy-turvy.

Women found with common aggressive breast cancer are often given a mastectomy, which is followed by other treatments. But women with inflammatory breast cancer typically undergo months of chemotherapy before having a mastectomy. The initial treatment helps relieve swelling and define clear boundaries between cancerous and healthy tissue.

That was the case for Kristine Turck, 39, of Yakima, who first noticed she had a retracted nipple in the summer of 2003. Turck has a health-care background, having trained as a psychiatric technician in California, but like so many others, she had never heard of the disease before she was diagnosed.

She endured eight months of chemotherapy before having a mastectomy in May 2004. The following month, she began a six-week course of radiation therapy. Doctors continue to treat her now.

Turck was administered radioactive dye that indicated her cancer had spread to her liver. She has not had any recent flare-ups. Turck said she has never asked for a prognosis because it won't change the outcome. However, she has changed the way she lives.

"I have learned to slow down," Turck said. "I used to run through life at a breakneck speed and not stop and relax."

Nancy Key, who also has not experienced recent flare-ups, said her diagnosis changed her lifestyle, too. She initially called friends and family to tell them how much she loves them. Then she and her husband, who dreamed of living on an island when they retired, moved to Camano Island.

In 2002, she was asked to join a peer-review panel that critiques how to spend the $150 million that Congress allocates each year toward breast-cancer research.

"I would not have attempted it before," Key said. "However, now I have a cause."

Nick Perry: 206-515-5639 or nperry@seattletimes.com

Copyright © 2004 The Seattle Times Company

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