Inflammatory breast cancer

Inflammatory breast cancer is the most aggressive form of breast cancer. Fortunately, it’s a rare form of breast cancer. Dr Daleen Geldenhuys elaborates.

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Inflammatory breast cancer (IBC) accounts for 0,5 to 2% of invasive breast cancers but contributes to 7% of breast cancer mortality. The median overall survival among women is less than four years, even with multi-modality treatment options. 

Patients with IBC usually present with breast pain or a rapidly growing, self-diagnosed breast lump. Patients may also complain of a tender, firm, warm-to-the-touch enlarged breast, or itching of the breast. 

IBC is more common in younger women (under age 40) and women who have an increased body mass index.

It can easily be confused with a breast infection, which is a much more common cause of breast redness and swelling. On presentation, almost all women with IBC have lymph node involvement, and approximately one-third have distant metastases.

IBC tends to have a higher incidence of major organ involvement, such as lung or liver metastases, compared with other forms of breast cancer. It metastasises earlier via the blood and lymphatic system. 

According to the TNM staging system where T indicates the size of the tumour; this tumour is designated as a T4d which immediately makes it a Stage 3 cancer (N is for nodal involvement and M for metastases).

Diagnosis criteria

All the following criteria must be met for diagnosis of IBC:

• Rapid onset of breast erythema (redness), oedema (swelling) and/or peau d’orange (dimpled orange peel skin) and/or warm breast, with or without an underlying palpable mass.
• Duration of history no more than six months.
• Erythema occupying at least one-third of the breast.
• Pathologic (biopsy proven) confirmation of invasive carcinoma. 

Histology

The classic histologic (seen under a microscope) finding in IBC on biopsy of affected skin is skin lymphatic channels packed by tumour cells. 

These malignant cells form tumour emboli (clots) which are responsible for both the local signs and symptoms (blocks lymph drainage so skin feels thicker) and for the development of metastatic disease. 

Thus, the term inflammatory breast cancer is a misnomer because there are no inflammatory cells in the lymphatic channels (white blood cells) but blockage of lymphatic channels by tumour cells. There are more genetic changes seen in the tumour cells of IBC than other breast cancers.

Primary IBC is not a specific histologic subtype of breast cancer, but the tumour is often of the ductal type. It can be HER2 positive or negative. Most of these tumours are hormone-receptor negative.

Improved prognosis

IBC is associated with a particularly poor prognosis and a high-risk of early recurrence. With multi-modality treatment, such as neoadjuvant chemotherapy (before surgery) and adjuvant chemotherapy (after surgery) and HER2 therapy if indicated, surgery and radiation, the survival rate is much higher than in the past. Five-year survival rates range between 30 and 70%.

Neoadjuvant therapy reduces tumour burden and makes surgery easier. It allows for less extensive surgery and improves cosmetic outcomes. It also permits evaluation of the effectiveness of chemotherapy, which the pathologist will comment on. The pathology report will guide the oncologist to make adjuvant therapy recommendations. The presence or absence of residual disease is also a prognostic factor for risk of recurrence.

The most important key to survival of any tumour, but especially IBC, is to pay attention to your body and do not delay going to a doctor if you notice any of the symptoms.

Header image by Adocbe Stock