Inflammatory Breast Cancer: The Disease, the Biology, the Treatment

Abstract

Inflammatory breast cancer (IBC) is a rare and aggressive form of invasive breast cancer accounting for 2.5% of all breast cancer cases. It is characterized by rapid progression, local and distant metastases, younger age of onset, and lower overall survival compared with other breast cancers. Historically, IBC is a lethal disease with less than a 5% survival rate beyond 5 years when treated with surgery or radiation therapy. Because of its rarity, IBC is often misdiagnosed as mastitis or generalized dermatitis. This review examines IBC's unique clinical presentation, pathology, epidemiology, imaging, and biology and details current multidisciplinary management of the disease, which comprises systemic therapy, surgery, and radiation therapy. CA Cancer J Clin 2010;60:351-375.© 2010 American Cancer Society, Inc.To earn free CME credit or nursing contact hours for successfully completing the online quiz based on this article, go to http://CME.AmCancerSoc.org.Inflammatory breast cancer (IBC) is a clinicopathological entity characterized by rapid progression and aggressive behavior from onset of disease. Historically, its prognosis has been very grim. Especially before the introduction of systemic chemotherapy, attempts to control IBC with either surgery alone or surgery combined with radiation therapy resulted in median survival times of less than 15 months and local recurrence rates as high as 50%. 1 Although survival times have increased with multimodal therapy, they are still around 35% to 40% and much lower than those for other breast cancers. Because IBC is rare, clinicians are less familiar with it than with the more common types of noninflammatory breast cancers (non-IBC). The purpose of this review is to describe the clinical diagnosis, epidemiology, imaging, biology, and multidisciplinary treatment of IBC. We summarize both current practice and novel concepts under investigation.

Radiological and clinical features of adult non-puerperal mastitis

“…Lesions were mammographically undetectable in 6 of 25 females, possibly because of an overlying dense mammary gland pattern observed in most females. With mammography, two patients in our study showed nipple retraction, and one showed skin thickening in the mammary areola, which is more commonly observed in inflammatory breast carcinoma or invasive carcinoma [7][8][9]. The ultrasound manifestations of non-puerperal mastitis varied.…”

“…This type of breast cancer is usually poorly differentiated in histology from infiltrating ductal carcinoma, which often has distant metastases at the time of diagnosis [8,26]. These aforementioned manifestations in non-puerperal mastitis are slight or rare [8]. Inflammatory breast cancer usually affects females of approximately 50 years old [26,27], but non-puerperal mastitis is more common in females under 40 [5,10,11,13].…”

“…Clinically, inflammatory breast cancer is characterised by the rapid onset of swelling and enlargement of the breast, the characteristic "peau d'orange" (orange peel) skin appearance and quick progression to nodules and ulcerations. This type of breast cancer is usually poorly differentiated in histology from infiltrating ductal carcinoma, which often has distant metastases at the time of diagnosis [8,26]. These aforementioned manifestations in non-puerperal mastitis are slight or rare [8].…”

“…The majority of patients with non-puerperal mastitis usually exhibit one or several breast masses without any other signs of inflammation. Because it can be very difficult to distinguish these symptoms from breast carcinoma, these patients can easily be misdiagnosed as having an inflammatory breast carcinoma or an invasive carcinoma [7][8][9].…”

Anoikis evasion in inflammatory breast cancer cells is mediated by Bim-EL sequestration

“…[1][2][3][4] This poor prognosis can be attributed to a number of factors, including the propensity for misdiagnosis of the disease due to its unique clinical presentation. [5][6][7] In contrast to most breast cancers, IBC is characterized by the lack of discernible primary tumor formation and the accumulation of cancerous epithelial cells in the dermal lymphatic vessels. 8 This lodging of IBC cells in the dermal lymphatics manifests as what appears to be inflammation, oftentimes causing clinicians to incorrectly diagnose the malady.…”