Inflammatory breast cancer (IBC) is one of the most aggressive forms of breast cancer.

And while certain types of breast cancer are widely known, many people have never heard of IBC – so what is it?

IBC is often referred to as an “orphan” disease because it is so rare, representing just one to three percent of all breast cancer cases in the U.S., according to the National Cancer Institute.

The complex disease, which is also aggressive and sometimes terminal, differs from other types of breast cancer, particularly in its symptoms, rapid evolution, prognosis and treatment.

IBC is typically:

  • diagnosed at younger ages, compared with other types of breast cancer
  • more common and diagnosed at younger ages in African American women than white women
  • more prevalent in obese women

The disease is triggered by cancer cells that block the lymph vessels in the skin of the breast, causing the breast to become swollen, red or inflamed.

Classical symptoms include pitting or ridging of the skin – making the breast appear like an orange peel – as well as tenderness, pain or itchiness.

The affected breast can swell, becoming larger, and might include a feeling of warmth and be physically heavier.

It’s important to note that if you experience these symptoms, it does not necessarily mean you have IBC. Because it’s difficult to differentiate between an infection and more serious issues, you should consult a physician immediately.

Given that symptoms can be falsely attributed, IBC can be difficult to properly diagnose.

It is not always characterized by lumps or tumors in the breast that are more easily discovered by a physical exam or mammogram.

In fact, a mass is typically discovered in mammography in only 10 percent of cases.

At diagnosis, IBC is minimum stage III, meaning it might have spread to nearby lymph nodes and skin surrounding the breast.

Undetected, it can spread to other parts of the body, affecting its ability to be treated effectively and promote positive survival outcomes.

For these reasons, affected women and physicians need to act swiftly if they recognize symptoms. Once detected, physicians can take advantage of different diagnostic tools, including a mammogram, breast ultrasound, MRI scan, CT scan or PET scan in order to investigate fully.

In most instances, treatment begins with chemotherapy followed by surgery and radiation therapy.

A treatment plan might also include a carefully selected standard of care treatment and-or clinical trials in areas such as immunotherapy, genomically targeted chemotherapy and other innovative research.

Dr. Ricardo H. Alvarez is a medical oncologist at Cancer Treatment Centers of America at Southeastern Regional Medical Center. He serves as medical director of the Breast Center and Director of Cancer Research.