Paget Disease of the Breast
Paget disease is a rare form of breast cancer, making up 1-4% of all breast cancer cases, according to the National Cancer Institute. The disease first appears on the nipple and often extends to the areola. In many patients, Paget disease may also be associated with a tumor or tumors in the affected breast.
Symptoms of Paget disease of the breast include:
- Eczema-like skin changes of the nipple and/or areola, including skin crusting, thickening and flakiness
- An ulcer on the nipple and/or areola
- Tingling or burning of the nipple and/or areola
- A flattened or inverted nipple
- Yellow or bloody nipple discharge
- A lump or lumps in the same breast as the affected nipple.
Many of these symptoms can be confused with skin conditions like eczema or dermatitis. As a result, many people show signs of Paget disease for several months before it is diagnosed. Partly due to these later-than-normal diagnoses, the survival rate for Paget disease is slightly lower than for breast cancer overall. However, if the disease is caught early, the chances for a cure are high.
If a doctor suspects a patient has Paget disease of the breast, the breast should be imaged. This can be done by a mammogram or possibly an ultrasound. If anything suspicious shows up in the image, such as a lump or calcified tissue, a small sample of the tissue is retrieved. This is often done during the imaging exam. From there, a pathologist will study the tissue sample in order to make a diagnosis.
If the images and biopsy comes back clear, though, the patient may still have cancer. At this point, the nipple and/or areola must be biopsied directly.
Treatment for Paget disease of the breast is similar to treatment for other breast cancers. The main difference occurs during surgery. When a patient undergoes surgery for Paget disease, whether a lumpectomy or mastectomy, the procedure requires the removal of the nipple and areola.
Why choose MD Anderson for Paget disease care?
Because Paget disease is a rare breast cancer, finding a team with the expertise to properly diagnose and treat this cancer is essential. The Nellie B. Connally Breast Center at MD Anderson is one of the largest and most active breast centers in the world, giving our team experience in treating Paget that few can rival.
Paget disease patients will also benefit from our multidisciplinary approach to care. All MD Anderson breast cancer patients have a breast surgeon, breast medical oncologist and breast radiation oncologist assigned to their case. These experts work together to develop a coordinated, personalized treatment plan for everyone they see.
This care is also available beyond MD Anderson¡¯s campus in the Texas Medical Center. Through our five Houston-area locations, patients throughout the region can get the same top-ranked care and personalized attention close to home.
Related Resources
Extramammary Paget disease (EMPD): What you need to know
Extramammary Paget disease (EMPD)?is a very rare form of skin cancer, affecting an estimated one in a million people in the U.S. It tends to grow slowly, but if left untreated, it can become invasive and spread to other parts of the body. That¡¯s why early detection is so important.
Because it¡¯s so rare, many doctors may never see a case in their careers. To help us understand more, we spoke with , a dermatopathologist who specializes in rare skin cancers like EMPD.
What is extramammary Paget disease?
EMPD is similar at the cellular level to a cancer called Paget¡¯s disease of the breast. When it appears outside the breast ¡ª on areas like the genitals, anus or armpits ¡ª it¡¯s called?extramammary, meaning ¡°outside the breast.¡±
What are the symptoms of extramammary Paget disease?
EMPD can look like many common skin conditions. It often appears as a red, scaly or irritated patch of skin. It may itch or hurt ¡ª or it may not cause any discomfort at all. Because of this, it¡¯s often mistaken for things like eczema, psoriasis or a fungal infection. Some people go months or even years before getting the correct diagnosis.
If a skin condition doesn¡¯t improve with standard treatments, it¡¯s worth asking your doctor to take a closer look.
Where does extramammary Paget disease usually appear?
Extramammary Paget disease typically shows up in areas with many sweat glands, such as:
- Women: Most commonly the vulva
- Men: Scrotum and penis
- Both: Around the anus, perineum or armpits
Are there different types of extramammary Paget disease?
Yes. There are two types of extramammary Paget disease:
- Primary EMPD starts in the skin or mucous membranes and stays there.
- Secondary EMPD is linked to internal cancers, such as rectal, bladder or prostate cancer in men, and cervical, ovarian or endometrial cancer in women.
About one-third of EMPD cases are secondary, so doctors will usually screen for internal cancers when EMPD is diagnosed.
How is Extramammary Paget disease diagnosed?
If a doctor suspects EMPD, they¡¯ll usually perform a?biopsy, removing a small piece of skin to examine under a microscope. Because EMPD can resemble other skin cancers, special tests (called?immunohistochemical stains) are often used to confirm the diagnosis and check for any deeper spread.
If EMPD is confirmed, additional tests may be needed to see if it has spread or if there¡¯s an internal cancer involved.