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Inflammatory breast cancer

Inflammatory breast cancer (IBC) is a rare cancer that gets its name from the swollen and red or “inflamed” appearance of the breast. Although quite rare, IBC is a very aggressive type of breast cancer in which the cancer cells block the lymph vessels in the skin of the breast. In the US, IBC accounts for up to 5 percent of all breast cancer cases.

Typically diagnosed in younger women than other forms of breast cancer, IBC is more likely to spread (metastasize) and its patients have a very low survival rate. About 70 percent of all women with the disease do not live five years beyond their diagnosis.

Causes

Despite its name, IBC isn’t caused by an inflammation or infection. It occurs when cancer cells clog the lymphatic vessels in the skin overlying the breast. The blockage in the lymphatic vessels causes the red, swollen and dimpled skin that’s a classic sign of IBC.

Signs and symptoms

Inflammatory breast cancer grows rapidly — changes can become apparent in a matter of days to weeks. And unlike other types of breast cancer, IBC probably won’t develop a distinct lump. It’ is also easy to confuse the signs and symptoms of IBC with those of a breast infection (mastitis). In some cases, redness, warmth and swelling of the breast are caused by a previous operation or radiation therapy that involved the outer breast or underarm, and not IBC.

Signs and symptoms of IBC include:

  • A breast that appears red, purple, pink or bruised
  • A tender, firm and swollen breast
  • A warm feeling in the breast
  • Itching of the breast
  • Pain, heaviness, burning and aching
  • Ridged or dimpled skin texture, similar to an orange peel
  • Thickened areas of skin
  • Enlarged lymph nodes under the arm, above the collarbone or below the collarbone
  • Flattening or retraction of the nipple
  • Swollen or crusted skin on the nipple
  • Change in color of the skin around the nipple (areola)

How is IBC diagnosed?

Diagnosis of IBC is usually based on the results of a doctor’s clinical examination. Biopsy, mammogram an breast ultrasound are also used to confirm the diagnosis. IBC is classified as either stage IIIB or IV breast cancer.

How is IBC treated?

Treatment consists of chemotherapy, targeted therapy, surgery, radiation therapy and hormonal therapy. Chemotherapy is generally the first treatment for patients with IBC. After chemotherapy, patients with IBC may undergo surgery and radiation therapy. Additional systemic treatments such as chemotherapy, hormonal therapy, targeted therapy or all three may also be given to reduce the risk of recurrence.

What is the outcome for patients with IBC?

IBC is more likely to have spread to other areas of the body at the time of diagnosis and as a result, the 5-year survival rate of IBC patients is only between 25 and 50 percent, which is significantly lower than the survival rate for patients with non-IBC breast cancer.

Paget’s disease

Paget’s disease of the nipple, also called Paget’s disease of the breast, is an uncommon type of cancer that forms in or around the nipple. It is an eczema-like change in the skin of the nipple, and 9 out of 10 women who have this have an underlying breast cancer.

Paget’s disease occurs in about 1–2 out of every 100 women with breast cancer. It usually occurs in women in their 50s, but can occur at an earlier or later age. It can affect men but this is extremely rare.

Signs and symptoms

  • Redness, inflammation and mild scaling or flaking of the nipple skin that won’t go away and may become sore.
  • There may also be crusting, slight bleeding and ulceration around the area.
  • In the more advanced stages of the disease, symptoms may include tingling, itching, increased sensitivity, burning, pain and discharge from the nipple.
  • Around half of the women who have Paget’s disease will have a breast lump that can be felt at the time it is diagnosed.

Causes

The cause of Paget’s disease is unknown, but two major theories have been suggested for how it develops.

One theory proposes that cancer cells, called Paget cells, break off from a tumor inside the breast and move through the milk ducts to the surface of the nipple, resulting in Paget’s disease of the nipple. This theory is supported by the fact that more than 97 percent of patients with Paget’s disease also have underlying invasive breast cancer or ductal carcinoma in situ (DCIS), a condition in which abnormal cells are present only in the lining of the milk ducts in the breast, and have not invaded surrounding tissue or spread to the lymph nodes.

The other theory suggests that skin cells of the nipple spontaneously become Paget cells. This theory is supported by the rare cases of Paget’s disease in which there is no underlying breast cancer, and the cases in which the underlying breast cancer is found to be a separate tumor from the Paget’s disease.

Diagnosis

Paget’s disease can be confused with other skin conditions such as eczema, dermatitis or psoriasis, as they can look very similar. This can make Paget’s disease difficult to diagnose.

Several tests may be carried out to diagnose Paget’s disease of the breast. They may include:

  • Mammogram
  • Ultrasound scan
  • Biopsy
  • Imprint or scrape cytology

Treatment

The treatment of Paget’s disease of the breast will depend on:

  • whether or not there is an underlying breast cancer.
  • whether it is DCIS or an invasive tumor.
  • how much of the breast is affected.

Surgery is the most common treatment for Paget’s disease of the nipple. The specific treatment often depends on the characteristics of the underlying breast cancer. Removal of the breast (mastectomy) may be recommended, especially if the cancer is affecting a wide area (is extensive), is close to the nipple, or if there is DCIS in a number of areas in the breast (multi-focal). Some of the lymph nodes in the under-arm area may also be removed.

Alternatively, patients whose disease is confined to the nipple and the surrounding area may undergo a lumpectomy followed by radiation therapy. In most cases, radiation therapy is commonly used as an adjuvant therapy to help prevent recurrence (return of the cancer). For some people, no further treatment will be needed after surgery. Others may need to have radiotherapy, hormonal therapy or chemotherapy. These may be used separately or together.

Emerging areas of diagnosis

Research is continuing into ways in which faulty genes increase the risk of breast cancer in some women. There are also many studies going on into diet and lifestyle and how those factors can play a role in preventing breast cancer.

Early diagnosis is a crucial part in the fight against breast cancer. For this reason, plenty of research is currently focused on breast screening procedures, such as:

Intraductal screening

An innovative way of detecting breast cancer, intraductal screening includes tests that get samples of fluid from the milk duct. The fluid in the ducts is in direct contact with the cells lining the breast ducts and lobules. This lining is a common place for breast cancer to develop. The breast cells in these ducts always produce a certain amount of fluid, even when a woman is not producing milk. The 3 tests that are being looked at are nipple aspiration, intraductal lavage and duct endoscopy.

Researchers have yet determine the reliability of intraductal screening in picking up cancer, or whether it can help determine a woman’s risk of developing breast cancer in the future. Clinical trials are still ongoing in the UK for intraductal screening.

Digital mammograms

This is a new way of recording mammograms. Instead of putting them on X-ray film, a computer is used to create and store the scans. X-rays are used to produce both digital and standard mammograms. As far as having the screening is concerned, there isn’t much difference between the 2 tests. The breast still has to be pressed down before the X-ray is taken.

Digital mammography is being tested in the US to see if it is any better at picking up breast cancers than film mammography.

MRI scans

Magnetic resonance imaging (MRI) is one of the methods used for diagnosing cancer, but doctors are still unsure about how useful it is for breast cancer. An ongoing trial is comparing MRI with mammogram and breast ultrasound. One of the aims of this trial is to find out if MRI provides any extra information for doctors before surgery.