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Paget’s Disease of the Breast

Paget’s disease of the breast is a rare form of breast cancer that starts on the nipple and extends to the areola (the outer dark circle of skin below the nipple). Compared with other types of breast cancer, Page’s disease only affects less than 5% women. It can also affect men, but this is very rare.

Symptoms of Paget’s Disease of the Breast

Although doctors are not yet aware of what causes Paget’s disease of the breast, most of the times, it presents itself like a skin irritation on the nipple similar to eczema or psoriasis. Other symptoms of Paget’s include:

  1. Itchiness of the nipple
  2. Flaky skin on the nipple
  3. Crusty and oozing skin on the nipple
  4. Burning sensation of the skin
  5. Nipple inversion or flattened
  6. Thickened skin on the breast
  7. A lump in the breast
  8. Bloody nipple discharge
  9. Tingling sensation on the breast

The difference between paget’s disease of the breast and eczema

As mentioned, Paget’s may look similar to skin conditions like eczema and psoriasis due to its flaky nature, but there is a major difference. Paget’s usually affects one breast, while eczema and psoriasis tend to affect both, and other parts of the body as well. Also, Paget’s affects the nipple, while these skin conditions affect the areola.

Diagnosis of Paget’s disease of the breast

Since Paget’s disease of the breast is very rare and can be confused with other skin conditions or rashes, it is generally not diagnosed straight away. Usually, it’s diagnosed through the doctor’s clinical judgement, after which he/she sends you to a specialist whereby a number of tests are ordered to arrive at a diagnosis. These tests include:

  1. Mammogram: This is a breast x-ray for possible identification of the cancer cells
  2. Ultrasound scan: An image of the cancer is produced using high frequency sound waves
  3. Biopsy: To confirm the diagnosis after undergoing the above tests, you will then have a biopsy. Here, a tissue sample is removed from the affected breast for analysis under a microscope. The tests will include scraping skin cells from the nipple, having a punch biopsy where a small amount of tissue is removed, and a core biopsy where another small sample of tissue from inside the breast.

Treatment of Paget’s Disease of the Breast

Treatment for Paget’s disease of the breast is usually dependent upon the stage of the cancer. After diagnosis, a customized treatment plan is prepared, with has different options.

The goal of the treatment is to get rid of all the cancer cells within the breast, outside and around the breast, as well as treatment of the entire body with specific drugs (systemic treatment) if needed. Depending on the condition or severity of the Paget’s disease, a combination of surgery, chemotherapy, targeted therapy, radiotherapy, hormone therapy, and bisphosphonates may be administered.

Surgery

Surgery is normally the first treatment of Paget’s disease. The surgery is done according to how the disease has progressed, the size of the affected area, and whether it has affected multiple areas on the breast.  You might have two types of surgeries:

  • Breast Conserving Surgery (BCS): Also known as a lumpectomy, the surgeon removes the cancer cells, as well as a margin of healthy breast tissue surrounding the tumor. During this procedure to treat Paget’s disease, the surgeon also removes the nipple and areola.
  • Mastectomy: This is the surgical removal of the entire breast, and it would be recommended if:
    • The area affected by Paget’s disease is large
    • The cancer cells are in multiple areas of the breast
    • The result of the lumpectomy will not give an acceptable cosmetic result

If one undergoes a mastectomy, and depending on each case, the patient might undergo a breast reconstruction surgery immediately after (immediate reconstruction), or at a later date (delayed reconstruction).

Lymph Node Removal

If the specialists find that there is an invasive form of breast cancer that’s underlying the Paget’s disease, then the next step would be to examine the lymph nodes under the arm to check for any signs of cancer. This is done through a lymph node biopsy, where a small sample of the lymph nodes are removed and tested for cancer cells. If the are found to be affected by cancer, then these lymph nodes are surgically removed – a procedure known as lymph node clearance.

Additional Treatments for Paget’s Disease of the Breast

After the lumpectomy or mastectomy, you may need additional treatments, known as adjuvant treatments. The goal is to give the best promising outcome. These treatments include:

1.      Chemotherapy

Chemotherapy treatment is a combination of anti-cancer cells used before surgery to either completely destroy or slow down the growth of the cancer cells throughout the body.

2.      Radiotherapy

Radiation therapy comes after chemotherapy and surgery with a goal of destroying any cancer cells and reduce the chances of it recurring. The highly controlled and carefully measured high energy x-rays target the entire breast, the lymph glands under the arm as well as those behind the breastbone.

3.      Hormone (Endocrine) therapy

Since some cancers can be stimulated by hormone estrogen, different hormone therapies can be used to stop the effect of estrogen hormone on cancerous cells. This mode of treatment is only considered if the Paget’s Disease of the Breast cancer cells test positive for receptors, which bind to the estrogen.

4.      Targeted (biological) therapy

If the Paget’s disease cancer cells have more than normal the amount of human epidermal growth factor receptor 2 gene (HER2), treatment drugs such as trastuzumab (Herceptin), which specifically targets the gene, may be given to treat it. And if the cancer tests positive for HER2, Pertuzumab (Perjeta), which is another targeted therapy, may be given. On the other hand, if the cancer is negative for HER2, then the drugs will not be administered.

5.      Bisphosphonates

Bisphosphonates are drugs that are used to either slow down or prevent bone damage. When used for cancer treatment in post-menopausal women, they reduce the risk of the Paget’s Disease of the Breast spreading.

Recovery After Paget’s Disease of the Breast

After the successful treatment of Paget’s Disease of the Breast, your doctor will keep a close watch on you. Follow-up appointments are required and should be followed as agreed with the breast doctor, where he/she will do tests for signs of recurrence. A mammogram and screening might be required every 6 to 12 months.

Next Steps

At King’s College Hospital London Dubai, our Breast Care Clinic caters to every type of breast cancer and breast conditions. If you are facing any kind of abnormal changes to your breast, regardless how small, get in touch for a consultation with one of our breast doctors. For more information on breast cancer and breast care, book an appointment below.

 

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