What is Paget’s disease of the breast?
Paget’s disease of the breast is an eczema-like rash on the skin of the nipple and is associated with an underlying breast cancer. It was described by Sir James Paget in 1874 who found that women who presented with this characteristic rash went on to develop breast cancer in the involved breast within two years. The underlying breast cancer may be an invasive breast cancer or ductal carcinoma in-situ (DCIS), meaning that the malignant cells have not yet invaded the surrounding tissue and are contained within the milk ducts of the breast.
Paget’s disease of the breast is rare and accounts for only 1-3% of new cases of breast cancer diagnosed annually in the United States. It is most commonly seen in women aged 50 -60, but it can occur in women younger or older, and even men, although this is extremely rare.
What causes it?
Unfortunately, doctors do not know what causes Paget’s disease of the breast. It is hypothesized that the disease results from an underlying breast cancer in the ducts, where the malignant cells travel through the milk ducts to the nipple and the surrounding skin.
What are the symptoms and signs of Paget’s disease of the breast?
Although Paget’s disease of the breast is an uncommon presentation of breast cancer, it is important that you contact your doctor if you have any of the following symptoms and signs as early detection can be life-saving.
· Scaly, flaky skin on the nipple
· Clear, yellowish discharge
· Bloody discharge
· Pain or pruritis (itchiness)
· Tingling or burning sensation
· Thickening of the skin
· Inverted nipple or retracted nipple
· Palpable lump in the breast
The signs and symptoms usually occur in one breast only. The disease usually starts in the nipple and then spreads to involve the areola, the surrounding dark area of the nipple. However, in rare cases, the areola may be the only skin affected. The skin changes seen can also fluctuate early on in the course of the disease, and it may even appear as though your skin is healing. However, if you are experiencing signs and symptoms for more than one month, see your doctor.
I have an appointment to see my doctor, what can I do to prepare?
You may be anxious to see your doctor and that is a completely understandable and normal feeling. When you see your doctor, he or she will inquire about the history of your present symptoms, personal medical history, family history and any risk factors you may have for breast cancer. Here are some things you can do to help prepare for your visit:
· List all the symptoms you are experiencing, when they started, and how long they have been present. Include ALL symptoms, even those that you may think are not related to the breast.
· Be familiar with your personal medical and surgical history, and family medical history.
· Make a list of medications you are taking, including vitamins and supplements.
· Write down any questions you may have, from most important to least important, so that you can address your concerns with your doctor during the appointment.
How is it diagnosed?
During your appointment, you doctor will want to perform a breast examination that will include inspection of both breasts as well as palpation for any lumps or abnormalities. Furthermore, because Paget’s disease of the breast can be confused with more common benign skin conditions such as eczema, psoriasis, or dermatitis, there are several tests that your doctor may perform to differentiate Paget’s disease of the breast:
· Mammogram (Breast X-ray): This test can help to see if the skin changes seen on the nipple are associated with an underlying breast cancer. If the results of the mammogram do not show any signs of a malignancy, your doctor may want to order magnetic resonance imaging (MRI) which may be able to detect cancer that was not seen on mammography.
· Ultrasound: A quick and easy test that can be done in the office. A gel is placed on the breast, and a small ultrasound probe is gently rubbed over the area, allowing visualization of the breast tissue by a computer monitor.
· Biopsy: A small sample of tissue from the skin of the nipple and any nipple discharge is collected and examined under a microscope. If there is a breast lump, a biopsy of that tissue will also be necessary. If cancer cells are detected, you may be referred to a breast surgeon to discuss treatment options (see below).
· Imprint or Scrape Cytology: Instead of a small tissue sample, cells from the affected areas of the nipple can be scraped, then pressed onto a glass slide for microscopic examination of malignant cells.
· Sentinel Lymph Node Biopsy: If there is invasive breast cancer, this procedure is done to see if the breast cancer has spread to the axillary lymph nodes, those located in the underams. This is a surgical procedure, in which the surgeon locates the sentinel nodes, i.e. the first lymph nodes that receive drainage from breast tumors and thus, where cancer cells will initially travel when spreading to other parts of the body. When a sentinel node is removed and found to be negative for cancer cells, then the likelihood of invasion to the axilla and other parts of the body is small. No additional lymph nodes need to be removed.
What are the treatment options?
You and your doctor together will come up with a treatment plan that fits your individual case. However, most cases of Paget’s disease of the breast requires surgery. The type of surgery needed will depend on how advanced the disease is – whether it is invasive or DCIS, and how much of the breast is affected:
· Simple mastectomy: Recommended if the underlying breast cancer has not spread to the lymph nodes. In this procedure, the entire breast is removed but the axillary lymph nodes (those located in your armpit which is the first site of metastasis) are left in place.
· Lumpectomy: This breast conserving surgery requires that you undergo adjuvant radiotherapy post-operatively. This procedure removes the nipple, areola, and the diseased portion of the breast, including a surrounding area of normal breast tissue, to insure that the cancer cells in its entirety is adequately removed. Most patients have nipple reconstruction after treatment.
After surgery, your doctor may recommend additional treatment, called adjuvant therapy:
· Radiotherapy: Typically employed after lumpectomy, radiotherapy uses high-energy x-rays to destroy cancer cells that may remain in the breast.
· Hormonal therapy. Mainly used if the cancer is invasive, but it may also be given to women who have DCIS. Tamoxifen and anastrozole are two commonly used hormonal treatments. They reduce the production of hormones to prevent stimulation of the cancer cells to grow.
· Chemotherapy. Cytotoxic (cancer cell-killing) drugs are used to destroy malignant cells. It is typically employed if the cancer is invasive.
Coping with breast cancer
Being diagnosed with breast cancer can be overwhelming. You may be feeling shocked, scared, guilty, angry, or anxious – all which are normal reactions and part of the process of accepting a new diagnosis. Every individual copes differently, but you may find it helpful in the beginning to speak openly with your doctor about the concerns you have and to get any additional information that may help you understand your illness better. Keep support systems around – talk with family and friends, find someone you can share your feelings with. You may find it beneficial to speak with breast cancer survivors – people who can truly empathize with you and understand wholeheartedly what you are going through during this difficult time. Most importantly, take care of yourself – eat healthy, exercise when you can, continue to partake in activities you enjoy, and get a good amount of rest.
Jamee Castillo, Shikha Jain MD