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Inflammatory breast cancer: Pathology and molecular pathogenesis

Sofia D Merajver, MD, PhD
Section Editor
Daniel F Hayes, MD
Deputy Editor
Sadhna R Vora, MD


Inflammatory breast cancer (IBC) is an aggressive form of locally advanced breast cancer. De novo IBC refers to primary disease. These patients typically present with pain and a tender, firm, and enlarged breast. The skin over the breast is reddened, warm, and thickened, with a "peau d'orange" (orange skin) appearance (picture 1). In comparison, the inflammatory recurrence of a noninflammatory breast cancer is called secondary disease. It usually develops on the chest wall at the site of previous mastectomy, but can also occur rarely as a distant cutaneous recurrence. The signs and symptoms of IBC arise rapidly compared to non IBC, typically within weeks to six months.

Primary IBC is a relatively rare disorder accounting for approximately 1 to 5 percent of invasive breast cancers [1,2]. However, because of its aggressive nature, it accounts for a greater proportion of cases presenting with more advanced disease. In one report of 752 patients with stage III breast cancer, for example, 24 percent had IBC [3].

At presentation, almost all women with primary IBC have lymph node involvement and approximately one-third have distant metastases. The long-term prognosis is also relatively poor, which has led to the development of combined modality treatment regimens consisting of neoadjuvant chemotherapy to maximize clinical response, followed by locoregional treatment, and then consolidation chemotherapy [4,5]. (See "Inflammatory breast cancer: Clinical features and treatment".)


The 2017 American Joint Committee on Cancer and the International Union for Cancer Control (AJCC-UICC) Tumor, Node, Metastasis (TNM) breast cancer staging system defines inflammatory breast cancer (IBC) as a clinical-pathologic entity characterized by diffuse erythema and edema (peau d'orange) involving a third or more of the skin of the breast (table 1). These "inflammatory" skin changes are not due to infiltration of inflammatory cells but rather to lymphedema caused by tumor emboli within the dermal lymphatics. However, the diagnosis is based upon the clinical presentation. Although dermal lymphatic involvement supports the diagnosis of inflammatory breast cancer, it is neither necessary nor sufficient in the absence of classical clinical findings.

IBC is designated as primary tumor stage T4d. Tumor presentation with ulceration and/or ipsilateral satellite skin nodules and/or edema are T4b. Not all skin changes qualify as IBC or even locally advanced breast cancer (LABC). Invasion of the dermis alone, such as dimpling of the skin or nipple retraction does not qualify as T4 and may occur in T1, T2, or T3 without changing the classification.

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Literature review current through: Nov 2017. | This topic last updated: Aug 14, 2017.
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