Systemic Therapy for Inflammatory Breast Cancer

Abstract

Inflammatory breast carcinoma (IBC) is the most aggressive, lethal, and rare form of breast cancer. IBC is characterized by the rapid development of erythema, edema, and peau d'orange over a third or more of the skin of the breast due to the occlusion of dermal mammary lymphatics by tumor emboli. IBC is associated with rapid progression, with a high risk of axillary lymph node involvement and distant metastases at initial diagnosis. The most striking progress in the management of IBC has been the sequential incorporation of preoperative systemic chemotherapy [an induction regimen containing an anthracycline and a taxane (plus trastuzumab in HER2-positive patients)], followed by surgery and radiation therapy. This multidisciplinary approach has essentially transformed IBC from a uniformly fatal disease with 5-year overall survival rates of less than 5% to one in which 5-year overall survival rates range from 46% to 61% and 15-year survival rates vary between 20% and 30%. The response to primary chemotherapy is a strong indicator of survival. The 15-year survival rate is 44% in patients who achieve a pathological complete response after primary chemotherapy. In this chapter, preoperative systemic therapy for the disease is discussed after a brief outline of inflammatory breast carcinoma.