Inflammatory breast cancer: An atypical breast cancer type

Once the diagnosis has been confirmed a variety of tests are needed for accurate treatment planning. IBC is always stage III or IV with approximately 30% of patients having metastatic disease at diagnosis. A positron emission tomography (PET); x-ray computed tomography (CT) of the chest, abdomen, and pelvis; as well as a bone scan are needed to assess the spread of the cancer. Staging and biomarker status from the biopsy tissue are essential for the development of a treatment plan.

Neoadjuvant chemotherapy is first administered to reduce tumor burden and convert the breast from inoperable to operable. In most cases, chemotherapy includes an anthracycline and a taxane; however, treatment regimens continue to change with the understanding and use of additional biomarkers to guide treatment. For example, the HER2/neu protein is often overexpressed in IBC, and can be targeted with a variety of anti-HER2 therapies. Such targeted therapy is often combined with chemotherapy and may be used in the neoadjuvant and adjuvant setting.

Hormone receptor status, the presence of estrogen and/or progesterone receptor activity in the tumor tissue, also impacts treatment. Tamoxifen can be used in premenopausal women to prevent estrogen from binding to its receptor, and aromatase inhibitors (such as letrozole) block the tumor’s ability to make estrogen. Both inhibit tumor growth in estrogen-dependent cancers. Ovarian suppression is also often recommended to reduce circulating estrogen levels among premenopausal women with IBC.

Knowing that not all patients have access to physicians with IBC experience, the NCCN developed a treatment guideline specific to IBC.⁹ Breast cancer guidelines version 2.2015 discusses the standard work-up following the pathologic diagnosis of IBC as well as recommended preoperative chemotherapy. These guidelines also discuss surgical management and radiotherapy.⁹

As noted in the NCCN Guidelines, a modified radical mastectomy is indicated for IBC due to the extensive nature of the disease within the breast. This surgery removes the entire breast and most of the lymph nodes from the affected axilla. The lack of a defined mass and extensive skin involvement eliminate the possibility of breast conserving therapy. In some cases, skin grafting may be necessary to obtain clear surgical margins. If there is a question of positive margins, patients should not undergo surgery but instead have a second-line chemotherapy or radiation prior to mastectomy. It should be noted here that immediate reconstruction is not recommended for IBC.

Radiotherapy plays an important role. Radiation to the chest wall and axilla after mastectomy helps kill cancer cells remaining in the skin and the lymphatic system of the muscle and soft tissue in the area. Radiation is typically administered daily for 6 to 7 weeks. A boost dose may be administered to the scar area following standard treatment. Other methods are often used to enhance the efficacy of radiation specifically for IBC.

In some cases, additional chemotherapy, targeted therapy, or antihormonal treatment will be given after surgery. Such treatment is used to help prevent disease recurrence. Importantly, care from the time of diagnosis should be based on the patient’s needs and concerns as well as to adequately manage side effects.

Clinical trial options are often limited for those with IBC, but nonetheless it can be useful for patients to consider a clinical trial. Patients may not be eligible for many trials once treatment begins; therefore, it is important to consider potential participation in clinical trials before initiating therapy.

The NCCN treatment guidelines can help the community oncologist manage the care of a patient with IBC; however, this is a very aggressive disease and may be better managed by a physician with IBC expertise. Several hospitals in the United States offer IBC-specific clinics, and most academic medical centers will have physicians with experience in the diagnosis and treatment of IBC.

Patients may receive more intense monitoring due to the aggressive nature of IBC. Specifics of disease monitoring will vary by physician and/or institution. As with standard breast cancer, patients with IBC will have regular follow-up with their surgeon, medical oncologist, and radiation oncologist. Patient-reported symptoms should be evaluated for potential disease recurrence.

Disease recurrences are a major concern in IBC. The most frequent sites of first recurrence are bone, central nervous system (CNS), lung/pleural effusion, liver, chest wall, and regional lymph nodes. Recurrence is more common in patients with triple negative (negative for estrogen receptors, progesterone receptors, and HER2) breast cancer (TNBC). Published data suggests that those with de novo stage IV disease have less CNS disease at diagnosis but may be at higher risk of CNS relapse. In spite of the high rate of CNS metastasis, routine CT or MRI screening of the brain remains controversial.⁶