Elsevier

Seminars in Oncology

Volume 35, Issue 1, February 2008, Pages 7-10
Seminars in Oncology

Inflammatory breast cancer
Defining the Clinical Diagnosis of Inflammatory Breast Cancer

https://doi.org/10.1053/j.seminoncol.2007.11.010Get rights and content

Inflammatory breast cancer (IBC) is an extremely aggressive disease that progresses rapidly and carries a very grim prognosis. It is characterized by erythema, rapid enlargement of the breast, skin ridging, and a characteristic peau d’orange appearance of the skin secondary to dermal lymphatic tumor involvement. Although a palpable tumor may not be present, about 55% to 85% of patients will present with metastases to the axillary or supraclavicular lymph nodes. Diagnosis of IBC is made on the basis of these clinical characteristics, as well as histopathologic verification of carcinoma. Accurate diagnosis is critically important, as multimodal therapy can significantly improve outcomes if instituted early enough.

Section snippets

Physical Appearance of the Breast

One of the earliest changes associated with IBC is erythema, with the skin overlying the breast showing a pink or mottled pink hue (Figure 2A). The color quickly changes to dark red or purple and spreads diffusely over the entire breast. The erythema may be associated with a sensation of heat in the affected breast. At about the same time, the breast begins to enlarge rapidly, sometimes increasing in size two- to threefold in a period of a few weeks (Figure 2B). This rapid rate of progression,

Differential Diagnosis

Conditions that are considered in the differential diagnosis of IBC are listed in Table 1. Bacterial infection, including mastitis and abscess, is a common misdiagnosis. However, these infections are rare in nonlactating women, and IBC is rare in young women of childbearing age, occurring more commonly in women 50 to 55 years of age. Duct ectasia (nonlactational chronic breast abscess) does occur in older women, but the area of erythema is generally well circumscribed and affects less than a

Screening for Metastasis

Because 20% to 35% of patients will have distant metastases at the time of presentation, additional tests are appropriate prior to the initiation of therapy in patients who have been diagnosed with IBC. The clinician taking the medical history and conducting the physical examination should be alert for any signs or symptoms of metastatic disease. Additional imaging, including a chest x-ray, bone scan, and computed tomography scan of the abdomen or sonography of the liver should be considered.

Conclusions

IBC is an aggressive cancer that must be diagnosed as early as possible to have the best chance of responding to aggressive multimodal treatment. Clinicians should be aware of this uncommon disease entity when assessing the patient who presents with rapidly developing diffuse erythema and swelling of the breast, even though they may lack the more common physical and mammographic indications for breast malignancy. Early assessment of imaging and confirmation of pathological diagnosis are

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    Clinically, IBC (Dabi et al., 2017; Van Uden et al., 2015; Woodward, 2015) and IMC (Clemente et al., 2010a; Peña et al., 2003; Pérez Alenza et al., 2001; Raposo et al., 2017) have a fulminant clinical course with rapid onset and are characterized by skin erythema, induration and edema of the mammary region and a short survival time after diagnosis. Histopathologically, the main criterium for histological diagnosis of IBC and IMC is the massive invasion of superficial dermal lymphatic vessels by neoplastic emboli with blockage of the lymphatic drainage causing the distinctive edema (Clemente et al., 2010b; Giordano and Hortobagyi, 2003; Pérez Alenza et al., 2001; Singletary and Cristofanilli, 2008; Ueno et al., 2018). Exacerbated angiogenesis, lymphangiogenesis, lymphangiotropism, vasculogenic mimicry (VM) and overexpression of COX-2, VEGF-A, and VEGF-D are characteristically found in IBC and IMC (Clemente et al., 2010a; Clemente et al., 2013; Peña et al., 2003; Van der Auwera et al., 2004).

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