In Paget disease of the breast, the skin around the nipple and the areola (the dark circle around the nipple) is affected. It is sometimes called Paget disease of the nipple or mammary Paget disease. In most cases, Paget disease affects women over 50, but it can also affect younger women and men. Paget disease of the breast often occurs in conjunction with an underlying breast tumor, which may exist in one breast or in the other. The nipple or the areola of the breast may be itchy, red, scaling, crusting, or bleeding due to Paget disease. Depending on the stage and type of the breast cancer, Paget disease can be treated with surgery, radiation therapy, chemotherapy, or hormone therapy. If you perform regular breast self-exams, get annual mammograms, and report any changes or symptoms to your doctor, you can prevent Paget disease of the breast.
There are two main theories regarding how Paget disease of the breast develops, but no exact cause is known. It is believed to be related to the abnormal growth of cancer cells in the breast.
A breast tumor's cancer cells travel through the milk ducts and multiply in the nipple and areola.
Cancer cells originate in the skin cells of the nipple and the areola, then spread to the underlying breast tissue.
The following factors may increase the risk of developing Paget disease of the breast:
In older women, especially after menopause, Paget disease of the breast is more common.
Women are more likely to develop Paget disease of the breast than men, but men can also develop it.
In families with a history of breast cancer or other types of cancer, Paget disease of the breast may run in the family.
There is a possibility that Paget disease of the breast may be associated with certain genetic mutations, such as BRCA1 or BRCA2, that increase the risk of breast cancer.
A hormonal change, such as pregnancy, breastfeeding, or hormone replacement therapy, may influence Paget disease of the breast.
The symptoms of Paget disease of the breast vary depending on the individual and the extent of the disease. The most common symptom is a change in the appearance or sensation of the nipple or the areola, including:
Pain, tingling, or itching
A rash, redness, or inflammation
Flaking, scaling, or crusting
A bleeding, oozing, or ulcerated wound
Nipple inversion or retraction
Nipple discharge or secretion
Paget disease of the breast can also cause the following symptoms:
Breast lumps or masses under the arm
Breast swelling or enlargement
Breast shape or size changes
Breast skin that is dimpling or puckering
Asymmetry or irregularity of the nipple or areola
Medical history, physical examination, and diagnostic tests are used to diagnose Paget disease of the breast. During a physical examination, the doctor will ask the patient questions about his or her symptoms, the onset and duration of the symptoms, the patient's medical history, and family history.
Examining the nipple and areola for abnormalities, such as color, texture, or shape
Examining the breast and lymph nodes for lumps, masses, or tenderness
Checking the nipple discharge for signs of blood, infection, or cancer
Diagnostic tests may also be ordered by the doctor, such as:
An X-ray of the breast can detect any abnormalities, such as tumors, cysts, or calcifications, through a mammogram
Using ultrasound imaging, you can see the structure and movement of breast tissue and fluid in the breast
Breast MRI provides detailed and contrasted images of the breast tissue and blood vessels using magnetic resonance imaging
Biopsies are performed on the nipple, the areola, or the breast to detect any cancer cells under a microscope
Tests that measure hormones, proteins, or markers that may indicate breast cancer type or presence
The treatment of Paget disease of the breast depends on the stage and type of the breast cancer, as well as the patient's overall health and preference. The main goal of the treatment is to remove the cancer cells and prevent them from spreading or recurring. Treatment options include:
In surgery, the affected tissue and sometimes surrounding tissue are removed, such as:
A lumpectomy removes the nipple, the areola, and some of the breast tissue around them
An entire breast and sometimes lymph nodes under the arm are removed during a mastectomy
In breast reconstruction, implants or natural tissue from another part of the body are used to rebuild the breast shape and appearance
In radiation therapy, high-energy rays or particles are used to destroy or damage cancer cells, such as:
In external beam radiation therapy, rays or particles are delivered to the breast or the chest wall from outside the body
The internal radiation therapy involves placing a radioactive material near the cancer cells inside the breast or chest wall
Drugs that kill or stop the growth of cancer cells, such as chemotherapy:
In systemic chemotherapy, the drugs are delivered through the bloodstream to the entire body
In regional chemotherapy, the drugs are delivered directly to the affected area, such as the breast or chest wall
Using hormone therapy, which involves blocking or lowering the levels of hormones, such as estrogen or progesterone, that may stimulate the growth of cancer cells, such as:
A drug called Tamoxifen blocks estrogen receptors on cancer cells, preventing them from receiving signals from estrogen
A drug called an aromatase inhibitor reduces estrogen levels in the body by inhibiting the enzyme that converts other hormones into estrogen
Surgically removing the ovaries or using drugs to suppress their function are two ways to stop the ovaries from producing estrogen
Using drugs that target specific molecules or pathways involved in the growth or survival of cancer cells, such as:
Trastuzumab binds to the HER2 protein on cancer cells and prevents them from receiving growth signals
In combination with trastuzumab, pertuzumab blocks the HER2 protein on cancer cells and enhances its effect
A drug called lapatinib inhibits the activity of the HER2 protein and another protein called EGFR on cancer cells, stopping them from growing
There are a number of measures that can be taken to reduce the risk or severity of Paget disease of the breast, including:
Checking your breast, nipple, and areola regularly for any changes or abnormalities
Screening for breast cancer and Paget disease with annual mammograms
Getting an early diagnosis and treatment for any symptoms you may have by reporting them to your doctor
Preventing breast cancer or Paget disease by avoiding or limiting exposure to risk factors such as smoking, alcohol, obesity, and hormone replacement therapy
Maintaining a healthy weight and a strong immune system by eating a balanced diet rich in fruits, vegetables, whole grains, and lean proteins
Regular exercise improves blood circulation, lymphatic drainage, and hormone balance
