Inflammatory Breast Cancer: The Experience of Baylor University Medical Center at Dallas

Abstract

It is estimated that 207,090 women will be diagnosed with and 39,840 women will die of breast cancer in 2010 in the United States. Inflammatory breast cancer (IBC) accounts for approximately 1% to 6% of all breast cancer cases in the US (1, 2). This rare and aggressive form of breast cancer is diagnosed clinically by the rapid onset of diffuse erythema and edema (peau d'orange) of at least a third of the skin overlying the breast (3) (Figure ​(Figure11). Tumor emboli blocking dermal lymphatic channels lead to the characteristic “inflammatory” skin changes; however, this is not necessary to make the diagnosis (4). The primary tumor of IBC is classified as T4d by definition, even if no underlying palpable mass is present in the breast. Figure 1 Typical clinical appearance of inflammatory breast cancer. Women diagnosed with IBC have inferior survival outcomes compared with women with other forms of breast cancer. IBC patients tend to be younger, and IBC tumors are more likely to overexpress HER2 than non-IBC tumors (5). Hormone receptor negativity also occurs at a higher frequency in IBC tumors (6). At presentation, most women with IBC have lymph node involvement, and approximately one third have distant sites of disease (7, 8). Historically, attempts to treat IBC with surgery alone or surgery combined with radiation therapy resulted in median overall survival times of less than 15 months and local recurrence rates as high as 50% (9). The treatment of IBC has dramatically improved with the advent of multimodality therapy. Results from a large retrospective study of patients with IBC performed over a 20-year period demonstrated that initial treatment with an anthracycline-based regimen followed by local therapy resulted in 5 - and 10-year survival rates of 40% and 33%, respectively (10). The incorporation of taxanes has also been associated with higher pathologic complete response rates and better survival outcomes. According to data from the National Cancer Institute's Surveillance, Epidemiology, and End Results database, for women who were diagnosed with IBC between 1988 and 2001, the 5-year survival rate was approximately 40%. This compares with about 87% for all breast cancers combined. The National Comprehensive Cancer Network (NCCN) panel recommends preoperative chemotherapy with an anthracycline-based regimen with or without taxanes for the initial treatment of patients with IBC. Inclusion of trastuzumab in the chemotherapy regimen is recommended for patients with HER2-positive disease. Patients responding to preoperative treatment should then undergo mastectomy with axillary lymph node dissection. Any remaining planned chemotherapy should be completed after the mastectomy, followed sequentially by endocrine therapy in patients with hormone receptor–positive disease. Finally, postmastectomy chest wall and regional node irradiation is recommended after the completion of any planned chemotherapy. As a historical reference, the Baylor Sammons Cancer Center medical committee and breast site committee constructed IBC treatment guidelines in 1989 that are in all practical purposes identical to the current NCCN guidelines. In 2010, the joint medical committee at Baylor Sammons Cancer Center requested a review of the IBC cases seen at our institution from 2003 to 2009 to evaluate compliance with the aforementioned guidelines as well as measure Baylor Dallas' outcomes data in accordance with the National Cancer Data Base (NCDB). This report presents those findings.