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Inflammatory breast cancer (IBC) is an uncommon type of invasive breast cancer that typically makes the skin on the breast look red and feel warm. It also may give the breast skin a thick, pitted appearance that looks a lot like an orange peel. These changes are caused by cancer cells blocking lymph vessels in the skin.

Because inflammatory breast cancer has reached these lymph vessels and has caused changes in the skin, it is considered to be at least a stage III breast cancer when it is diagnosed. IBC that has spread to other parts of the body is considered stage IV. These cancers typically grow quickly and can be challenging to treat.

Regardless of the stage of the cancer, participation in a clinical trial of new treatments for IBC is also a good option because IBC is rare, has a poor prognosis (outcome), and these studies often allow access to drugs not available for standard treatment.

Treating stage III inflammatory breast cancer

IBC that has not spread outside the breast or to nearby lymph nodes is stage III. Treatment usually starts with chemotherapy (chemo) to try to shrink the tumor. If the cancer is HER2-positive, targeted therapy is given along with the chemo. This is typically followed by surgery (mastectomy and lymph node dissection) to remove the cancer. Radiation therapy often follows surgery. Sometimes, more chemo may be given after surgery but before radiation. If the cancer is hormone receptor-positive , hormone therapy is given as well (usually after all chemo has been given). Combining these treatments has improved survival significantly over the years.

Chemotherapy (possibly along with targeted therapy)

Using chemo before surgery is called neoadjuvant or preoperative treatment. Most women with IBC will receive two types of chemo drugs (although not always at the same time):

• An anthracycline, such as doxorubicin (Adriamycin) or epirubicin (Ellence)
• A taxane, such as paclitaxel (Taxol) or docetaxel (Taxotere)

Other chemo drugs may be used as well.

If the cancer is HER2-positive (the cancer cells make too much of a protein called HER2), the targeted therapy drug trastuzumab (Herceptin) is usually given, sometimes along with another targeted drug, pertuzumab (Perjeta). These drugs can lead to heart problems when given with an anthracycline, so one option is to give the anthracycline first (without trastuzumab or pertuzumab), followed by treatment with a taxane and trastuzumab (with or without pertuzumab).

For IBC that is triple-negative, the immunotherapy drug pembrolizumab can be given with chemo before surgery (neoadjuvant treatment) and then continued by itself after surgery (adjuvant treatment).

For women who have:

• a BRCA mutation AND
• triple-negative or HER2-negative IBC AND
• residual cancer in the tissue removed by surgery after neoadjuvant chemo

the targeted drug olaparib (Lynparza) might be given to lower the risk of the cancer recurring. It is typically given for one year. When given this way, olaparib can help some women live longer.

Surgery and further treatments

If the cancer improves with chemo, surgery is typically the next step. The standard operation is a modified radical mastectomy, where the entire breast and the lymph nodes under the arm are removed. Because IBC affects so much of the breast and skin, breast-conserving surgery (partial mastectomy or lumpectomy) and skin-sparing mastectomy are not options. It isn’t clear that sentinel lymph node biopsy (where only one or a few nodes are removed) is reliable in IBC, so it is also not an option.

If the cancer does not respond to chemo (and the breast is still very swollen and red), surgery cannot be done. Either other chemo drugs will be tried, or the breast may be treated with radiation. Then if the cancer responds (the breast shrinks and is no longer red), surgery may be an option.

If breast radiation isn’t given before surgery, it is given after surgery, even if no cancer is thought to remain. This is called adjuvant radiation. It lowers the chance that the cancer will come back. Radiation is usually given 5 days a week for 6 weeks, but in some cases a more intense treatment (twice a day) can be used instead. Depending on how much tumor was found in the breast after surgery, radiation might be delayed until further chemo and/or targeted therapy (such as trastuzumab) is given. If breast reconstruction is to be done, it is usually delayed until after the radiation therapy that most often follows surgery.

Treatment after surgery often includes additional (adjuvant) systemic treatment. This can include chemo, targeted therapy, hormone therapy (tamoxifen or an aromatase inhibitor) if the cancer cells have hormone receptors, the oral chemo drug capecitabine (Xeloda) if the cancer is triple-negative, the PARP inhibitor olaparib (Lynparza) if the woman has a BRCA mutation,and/or trastuzumab, pertuzumab or ado-trastuzumab emtansine if the cancer is HER2-positive.

Treating stage IV inflammatory breast cancer

Patients with metastatic (stage IV) IBC are treated with systemic therapy. This may include:

• Chemotherapy
• Hormonal therapy (if the cancer is hormone receptor-positive)
• Targeted therapy with a drug that targets HER2 (if the cancer is HER2-positive)
• Immunotherapy if the cancer makes a protein called PD-L1
• Targeted drug therapy with a PARP inhibitor called olaparib if the woman has a BRCA mutation

One or more of these treatments might be used. Many times, a targeted drug is given along with chemotherapy or with hormone therapy. Surgery and radiation may also be options in certain situations. See Treatment of Stage IV (Metastatic) Breast Cancer for more information.