One less common breast cancer is inflammatory breast cancer, or IBC, which is an aggressive form of the disease. The main warning signs of IBC are swelling and redness of the breast. Most IBC begins as invasive ductal carcinoma, which means that it starts in the milk ducts. About 1 to 5% of breast cancers in the U.S. are IBC. Black women and women who are obese are more likely to be diagnosed with IBC than other women. IBC can be hard to detect on a mammogram. It’s often spread throughout the breast, or it may only show up as a sign of inflammation, like skin thickening. Other breast changes may include dimpling or puckering of the skin, pulling in of the nipple and breast pain. Sometimes a lump can be felt, but it’s less common than with other breast cancers. These warning signs tend to happen quickly, within weeks or months. If you notice any of these changes and they last more than a week, contact your health care provider. Although IBC may be diagnosed based on how it looks, a biopsy is needed to confirm the diagnosis. About one third of women with IBC already have metastasis when they are first diagnosed, which means cancer has spread beyond the breast and nearby lymph nodes to other parts of the body. So, it’s common for tests to be done that check for metastatic breast cancer. Susan G. Komen, in collaboration with the Inflammatory Breast Cancer Research Foundation and the Milburn Foundation, brought together a team of leading breast cancer experts including clinicians, researchers and IBC patients to develop the IBC Scoring System. This tool for health care providers is intended to increase diagnostic accuracy, guide treatment decisions and inclusion in clinical trials, and aid basic research. IBC is treated with chemotherapy, surgery and radiation therapy. Depending on the characteristics of the tumor, treatment may also include hormone therapy, HER2- targeted therapy or other drug therapies. The first treatment for IBC is neoadjuvant chemotherapy, which helps shrink the tumors in the breast and lymph nodes, making them easier to remove during surgery. If the first combination of chemotherapy drugs doesn’t make the tumor smaller, other drug combinations can be tried.

Surgery is the next treatment, which is almost always as mastectomy with an axillary lymph node dissection. Surgery is followed by radiation therapy. If a person is having breast reconstruction, it’s usually done after radiation. Treatments given after surgery and radiation therapy depend on the treatments that were given before surgery and the characteristics of the tumor. Although survival rates for inflammatory breast cancer may not be as high as they are for other breast cancers, research is ongoing to improve treatment and survival. For more information, visit the Inflammatory Breast Cancer page on komen.org. Another rare type of breast cancer is metaplastic breast cancer, which makes up less than 1% of all invasive breast cancers. These tumors tend to be larger and have a higher grade than more common types of breast cancer. They’re also more likely to be triple negative, which means they are estrogen receptor-negative, progesterone receptor-negative and HER2-negative. Under a microscope, metaplastic breast cancer cells can look very different from tumor cells of more common cancers. Because of this, they’re often confused with other uncommon breast tumors or tumors from other parts of the body, making it harder to diagnose. For this reason, it’s often recommended that the pathology slides be reviewed by a second pathologist to confirm the diagnosis. For more information, visit the Metaplastic Breast Cancer section on komen.org. IBC, metaplastic breast cancers and others can be triple negative, which is not a type of tumor, but a subtype. Triple negative breast cancer, or TNBC, is negative for estrogen receptors, progesterone receptors and HER2. Being triple negative means the cancer cells don’t respond to certain therapies, such as hormone therapy. However, there are effective treatments available. About 15 to 20% of breast cancers are triple negative. TNBC is aggressive and tends to occur more often in younger women, people with a BRCA1 inherited gene mutation and Black women. It may also be more common in Hispanic women compared to white women. Since triple negative tumors are often aggressive, they’re more likely than estrogen receptor-positive breast cancers to recur or come back, at least within the first 5 years after diagnosis. After about 5 years, the risk of TNBC recurrence is lower. Early triple negative breast cancers are usually treated with some combination of surgery, radiation therapy, chemotherapy and sometimes immunotherapy.

Triple negative tumors can’t be treated with hormone therapy or HER2-targeted therapies. However, people with TNBC often benefit more from chemotherapy than people with hormone receptor-positive breast cancers. For more information, visit the Triple Negative Breast Cancer page on komen.org. Paget disease of the breast is a rare form of carcinoma in situ in the skin of the nipple or areola, which is the area of skin surrounding the nipple. In situ means the abnormal cells are only found in these places and have not spread. Warning signs and symptoms can include itching, burning, redness or scaling of the skin of the nipple or areola, a bloody or yellowish discharge and a flattened nipple. If you notice any of these changes, contact your health care provider. Paget disease of the breast can be diagnosed with a biopsy of the nipple skin and the tissue just beneath it. This can be done with a punch biopsy, or another type of biopsy. Paget disease is often found when an underlying breast cancer is diagnosed, which happens in about 1 to 4% of breast cancers. It may be found with invasive breast cancer or ductal carcinoma in situ, or DCIS, a non-invasive breast cancer. In these cases, prognosis and treatment depend on the underlying breast cancer. For more information, visit the Paget Disease of the Breast page on komen.org.