Epidemiology of Inflammatory Breast Cancer (IBC)1

Abstract

Inflammatory breast cancer (IBC) is a rare and aggressive form of breast cancer with unknown etiology and generally poor outcome. It is characterized by diffuse edema (peau d'orange) and redness (erythema), although either the disease itself or case definitions have varied over time and place, confounding temporal trends and geographic variations. In this review, we discuss case definitions for IBC and its clinical characteristics; describe its geographic variation, age and racial distribution, incidence and survival patterns, and summarize the very limited information on its epidemiologic risk factors. We also incorporate emerging data from the National Cancer Institute's (NCI) Surveillance, Epidemiology, and End Results (SEER) Program. KeywordsInflammatory breast cancer; epidemiology; etiologic factors Case Definition for Inflammatory Breast Cancer (IBC)IBC is an aggressive and lethal form of breast cancer. It is characterized by the clinical appearance of inflammation, with edema and redness of the breast. However, the appearance of inflammation is not due to true physiologic inflammatory response but rather due to pathologic plugging of the dermal lymphatics of the breast with tumor emboli. This condition was first described in 1814 by Sir Charles Bell [14]; but, it was not until 1924 that the term inflammatory breast cancer was proposed by Lee and Tannebaum at the suggestion of James Ewing [56,88]. Prior to this designation, IBC was referred to as von Volkmann's or Wokmann's syndrome in pregnant women, lactation cancer, carcinoma mastitoides, mastitis carcinomatosa, acute encephaloid cancer, acute mammary carcinoma, acute mammary carcinomatosis, acute brawny cancer, acute scirrhous carcinoma, acute medullary carcinoma or carcinoma telangiectaticum [42,53,55,57,71,75,82,90,92]. In all, these expressions implied an acute and aggressive breast cancer, more of a clinical than a pathological entity, often multifocal [13,18,19,33,38,66,75,92], and occurring in young women during pregnancy or lactation [53,55,75,90].The extent of clinical signs required for IBC has not been standardized and has varied from any edema (peau d'orange) and redness (erythema) to edema and redness covering the entire

Exploring patient experiences of neo-adjuvant chemotherapy for breast cancer

“…We did not collect data on stage of disease or type of breast cancer from patient medical notes so we do not know how many of our sample had inflammatory breast cancer, the most aggressive form of breast cancer that tends to affect younger women (Anderson, Schairera, Chena, Hance, Levine 2006). Given the young age of our sample it seems likely that some of these women had a diagnosis of inflammatory breast cancer; further work in this area should record histological details.…”

Cancer diagnosis in primary care

“…'Peau d'orange' changes in the skin are common. 21 These appear quite rapidly, making differentiation from infection difficult. The underlying pathology is blockage of the local lymph vessels by cancer cells, so the prognosis of this form of breast cancer is poorer.…”

MicroRNA expression profiling identifies decreased expression of miR-205 in inflammatory breast cancer

“…At the time of presentation, 55 to 85% of patients have regional lymph node metastasis clinically. 2 Despite improved survival in the past three decades because of the introduction of multimodality treatment approaches, the survival outcomes of patients with inflammatory breast cancer remain poorer compared with patients with noninflammatory, locally advanced breast cancer, 3,4 underscoring the need for understanding the biology of the disease and exploring new therapeutic targets.…”