My mother was diagnosed with inflammatory breast cancer (IBC) in 1981. IBC accounts for between 1 to 5 percent of all U.S. breast cancer cases–NIH classifies it as a rare disease. Although some small progress has been made since my mother died almost 30 years ago, it remains the most aggressive type of breast cancer.
It is estimated that between 1% and 5% of all newly diagnosed breast cancers each year present as IBC; because of its rarity, it is listed with the Office of Rare Diseases at the National Institutes of Health. While the number of cases of IBC is relatively small compared with the overall number of breast cancers, it is still a substantial number compared with many other rare tumor types…We cannot use the small number of cases as an excuse for the lack of clinical trials; rather, we should view it as a mandate for making novel treatment strategies available to all patients with this diagnosis.
IBC patients’ median ages range between 45 and 55 years old. It occurs more frequently and at a younger age in African Americans vs. Caucasians. (See Inflammatory Breast Cancer Foundation – What is IBC? as well as IBC Support.)
Dr. Naoto Ueno alerted me to this recent IBC article. Because we tend to read very little about this type of breast cancer, I wanted to share it. Some key point follow.
By Sunita Patterson
Inflammatory breast cancer (IBC) stands apart from other cancers of the breast in its unusual clinical presentation, its aggressiveness, and its poor prognosis. Researchers and clinicians are working to clarify what distinguishes IBC from other breast cancers and to discover treatments that improve patient outcomes.
Although IBC accounts for only 2%–5% of breast cancers, it is responsible for 8%–10% of breast cancer–related deaths. “IBC has a strong tendency to metastasize. In fact, a third of patients have metastases at diagnosis,” said Naoto T. Ueno, M.D., Ph.D., a professor in the Department of Breast Medical Oncology and executive director of the Morgan Welch Inflammatory Breast Cancer Research Program and Clinic at The University of Texas MD Anderson Cancer Center. The IBC program at MD Anderson was the first clinic in the world devoted to IBC and is the largest today, treating about 100 patients each year.
Symptoms and diagnosis
Because IBC is rare and its symptoms differ from those of more typical breast cancers, misdiagnosis and less-than-optimal treatment are common, both of which diminish survival outcomes.
The first challenge that IBC presents clinicians is that it does not look like a typical breast cancer. It often appears to be and is misdiagnosed as an infection or a rash. The primary symptoms are usually rapid breast enlargement and erythema covering most of the breast; there may not be any lump.
For most patients with breast irritation and redness, mastitis is the problem, and an antibiotic will help. However, according to Dr. Ueno, if there is no response to the antibiotic in 1–2 weeks and the breast remains red, the physician should suspect IBC and order a biopsy right away. “We don’t want to waste a lot of time with this disease,” Dr. Ueno said, “because it increases the chance of metastasis.”
Both a core needle biopsy and a punch biopsy of the skin should be done. When there is no clearly defined mass, Dr. Ueno recommends directing the needle where the most swelling and redness exist. In patients with IBC, the skin specimen will often show extensive dermal lymphatic invasion. However, in the presence of persistent symptoms, negative biopsies do not rule out cancer completely.
Along with the biopsies, the patient should undergo mammography. If the results are negative, magnetic resonance imaging and ultrasonography should be considered.
“Many community physicians will just see one case of IBC in their entire practice,” Dr. Ueno said. For this reason, he strongly recommends that patients with IBC be referred to a clinic specializing in IBC treatment. “These patients need a specific workup and a multidisciplinary care team,” he said.
The MD Anderson IBC clinic usually sees patients within 48 hours of their referral or self-referral. Typically, patients initially come to MD Anderson for 10–14 days of testing and meeting with medical, surgical, and radiation oncologists. The workup consists of repeat breast imaging (mammography, magnetic resonance imaging, and ultrasonography), remapping of the lymph nodes, blood tests, a pathologic review, and sometimes a positron emission tomography–computed tomography scan or a computed tomography scan with a bone scan.
Treatment of nonmetastatic IBC differs from that of other breast cancers in that systemic therapy is given preoperatively to debulk the disease as much as possible. Surgery and radiation therapy follow. Because treatment usually begins with chemotherapy, a medical oncologist is usually consulted first. But Dr. Ueno recommends that surgical and radiation oncologists with expertise in IBC also be involved from the beginning. “The optimal extent of local treatment can be difficult to judge when there isn’t a clear mass and the redness is diffuse,” he said, adding that coordinated care by an experienced team can reduce the possibility of errors.
Dr. Ueno is passionate about the need to study IBC even though it’s “rare.” “People keep asking, why are we investing so much effort in an ‘orphan’ disease?” he said. “It’s true that IBC is rare in terms of incidence. But from the mortality perspective, IBC isn’t rare. It’s a killer.”
For more information, call Dr. Naoto Ueno at 713-792-8754.
Dr. Naoto Ueno recently co-edited a textbook about IBC treatment and research: Inflammatory Breast Cancer: An Update (Ueno NT, Cristofanilli M, eds.). Springer, 2012.
The MD Anderson IBC program hosts a Web page, Facebook page, and Twitter account with research updates and patient information:
Dr. Ueno posts information for patients, clinicians, and researchers on Facebook and Twitter: