Inflammatory breast cancer: Researchers advocate for more understanding of rare malignancy

"Inflammatory breast cancer is a disease that needs more attention, and more education for physicians and patients," says Massimo Cristofanilli, M.D., F.A.C.P., chair of the department of medical oncology at Fox Chase Cancer Center and senior author of the review, which will appear in the Nov/Dec issue of CA: A Cancer Journal for Clinicians.

The American Cancer Society estimates that IBC accounts for 2.5% of all breast cancer cases in the United States, with 192,370 new diagnoses and 40,170 deaths expected this year. Yet Cristofanilli emphasizes that it is not necessary for physicians to have already seen a case of IBC to diagnose it properly. "If you know about it and you have a clinical suspicion, it is important to reach out to a specialist as soon as possible," he says.

Patients frequently come to their primary physician or gynecologists complaining of reddened skin and swollen breast, which might be initially mistaken for a rash or infection. "Ninety-five percent of women with IBC receive antibiotics for a while before they are correctly diagnosed," Cristofanilli says. "But it is important to recognize that you cannot have an infection without fever. If a woman is not nursing, is 55 years old, and has no other risk factors for mastitis, it is much more likely that she has a cancer than an infection."

And though many oncologists have often considered IBC a type of locally advanced breast cancer, the reality is that IBC is a distinct disease. "A typical advanced breast cancer has a mass or nodule that grows over months or years, and eventually involves most of the breast and could show also with some redness," Cristofanilli says. "With IBC, you have a tumor that becomes clinically evident in just a few weeks -- sometimes women describe it as just a few days. The breast is red and inflamed, but there is no fever; within a few days, the cancer is already in the lymph nodes and there is no palpable mass. So if you picture these two situations, you clearly see there is no resemblance."

The difference in presentation and symptoms suggests that IBC has a distinct underlying biology, relative to locally-advanced breast cancer. And many years of research have shown that IBC does not respond to therapies used to treat other forms of breast cancer.

New and more effective therapies are in the works though, according to Cristofanilli and first author Fredika Robertson, Ph.D., professor in the Department of Experimental Therapeutics at The University of Texas MD Anderson Cancer Center. Basic science researchers, such as Robertson, are pinpointing molecular pathways that drive the formation and growth of IBC, and new agents targeted against those pathways are in development.

For example, on-going clinical trials are testing lapatinib, a tyrosine kinase inhibitor that blocks two signaling pathways that are frequently hyperactive in IBC. "Lapatinib is one of the few drugs that shows activity as a single drug in IBC, but this is still an evolution," Cristofanilli says. "The paper addresses some of the areas where we expect to see novel agents come and the direction we will go in the next few years."

"I think the future is bright if we are all able to come together and recognize that there is a need to put resources and research into this disease," he says. "And many researchers are already doing this."