Inflammatory breast cancer (IBC) is a rare and aggressive subtype of locally advanced breast cancer (LABC). Its diagnosis is primarily clinical; however, a pathological confirmation of invasive cancer is required. Historically, IBC was a uniformly fatal disease. A major advance in the last three decades has been the introduction of a multidisciplinary approach to the management of this aggressive disease, incorporating pre-operative chemotherapy, surgery, and radiation therapy; this approach has significantly improved survival. Our review focuses on the progress made in the field of IBC research over the last decade, with particular attention to advances in the areas of epidemiology, molecular biology, and clinical management.
Impact of Advances on Survival Outcomes: What Progress Have We Made?
When looking at the survival outcomes of women with IBC, several questions need to be addressed. First, has the overall survival of women diagnosed with IBC improved? The answer to this question is a clear-cut yes. With the introduction of enhanced diagnostic techniques and sequential treatment with pre-operative chemotherapy, surgery, and radiation therapy, median overall survival has significantly improved from approximately 15 months to 40 months;[3, 35] studies document that approximately 28% of women with IBC are alive and free of disease at 15 years. Second, has there been a steady improvement in the survival of women with IBC over the past three decades? The answer to this question is not clear cut. Gonzalez-Angulo et al looked at whether the survival of women with IBC who were treated at the M.D. Anderson Cancer Center had improved over the past 30 years. The authors reported that in the multivariable models, after adjusting for a number of patient and tumor characteristics, increasing year of diagnosis was not associated with a decrease in either the risk of recurrence (hazard ratio [HR] =1.00; 95% CI = 0.97–1.04) or death (HR = 0.97; 95% CI = 0.94–1.01). However, the authors of the study acknowledged a number of limitations that could have contributed to the observed results. Third, is the survival of women with IBC in the 21st century similar to that of women with LABC? In a retrospective review of women in the Surveillance, Epidemiology and End Results (SEER) registry, our group has recently shown that women with IBC who were treated between 2004 and 2007 in settings where a multidisciplinary disciplinary approach to treatment is considered standard of care, continue to have poorer survival outcomes when compared with women with non-IBC LABC (personal communication with authors; paper in press).
Introducing the First Consensus Guidelines
It is clear that important and significant progress has been made in the field of IBC. However, there is still a long way to go. Despite an improved treatment approach that has had a positive impact on the prognostic outcome of this disease, the survival outcomes are still poor and still inferior to those of non-IBC LABC. The future of IBC will depend on a deeper understanding of the disease at the molecular level. The data available so far indicate that molecular subtypes defined within non-IBC tumors also exist within IBC tumors. Future research will need to concentrate on defining the prognostic and predictive value of these subtypes in IBC as well as identifying potential therapeutic targets.
One of the biggest challenges facing researchers in the field of IBC is the rarity of the disease; its rarity has an impact on both epidemiological and translational research. To address this issue, a group of international experts in December 2008 formed the first IBC consortium. This consortium formulated and recently published the first international guidelines on the diagnosis and management of IBC, with the objective of standardization. With the standardization of diagnostic criteria and therapeutic strategies in IBC, and with the consortium encouraging collaboration of research at both the clinical and molecular level, the next decade is bound to see further progress in the field of IBC.
Financial Disclosure: Dr. Dawood has received honoraria from Roche. Dr. Cristofanilli has no significant financial interest or other relationship with the manufacturers of any products or providers of any service mentioned in this article.
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