Inflammatory breast cancer: Where are we now?

Abstract

Inflammatory breast cancer is perhaps the most aggressive form of breast neoplasm, with a poor prognosis. Clinically, inflammatory breast cancer is characterized by erythema and edema of the skin of the breast, called "peau d`orange", with or without an associated palpable mass. This form represents 1% to 6% (doubled during the past two decades) of all newly diagnosed breast malignancies and is often considered together with local advanced breast cancer, despite specific differential features. The reported 5-year survival rates range from 10% to 75%. On mammography, a diffuse increase in density and skin thickening may be present. Pathologic confirmation of invasion of dermal lymphatics by malignant cells can help distinguish this condition from benign mastitis. Most inflammatory breast cancers are on biopsy poorly differentiated ductal carcinomas, mainly estrogen and progesterone receptor negative. HER2/neu-overexpression and p53 gene mutations are frequently present. The probability of axillary node involvement is approximately 90%. Contralateral breast cancer develops in 25% to 50% of the patients, usually in the presence of metastases. Inflammatory breast cancer has a high rate of locoregional recurrence after surgery and/or radiotherapy and the rapid appearance of distant metastases. However, long-term survival is possible for these patients if treated with multimodality therapy including polychemotherapy, mastectomy, and loco-regional radiotherapy. The optimal sequencing of combined modality therapy has not been determined. Although not all patients are candidates for such a regimen (often due to progression during treatment), for those that complete all phases of therapy, more than one-third will be alive without disease at 10 years.