Paget's Disease Of The Nipple
A Rare Expression of Breast Cancer

Paget's disease typically appears as a red, scaly rash around the nipple. Under the microscope, cells form in clusters near the surface.

Several years ago, there was an e-mail circulating on the Internet that began:

"In November I lost my sister (Betty Botts of Troy, Al.) to a rare kind of cancer. She developed a rash on her breast similar to that of young mothers who are nursing."

It goes on to send a harsh warning about Paget's Disease.

No one was able to verify if any of the women named in the e-mail exist. However, Paget's Disease is real. It is an extremely rare, and not normally deadly, form of breast cancer. It does need to be diagnosed and treated.

The course of the disease that the e-mail describes sounds much closer to the progression of Inflammatory Breast Cancer (IBC). IBC is also rare, but it is a very aggressive form of breast cancer, which also begins as what looks like a rash on the breast. IBC is always diagnosed at Stage III or Stage IV and needs to be treated quickly with the appropriate therapies to delay or prevent the spread to other organs.

What Is Paget's Disease of the Nipple?
The real name of the disease is "Paget's Disease of the Nipple," "Paget's Disease of the Breast" or "Mammary Paget's Disease."

It is named after the English surgeon, Sir James Paget, who first described it in the 1800s. Sir James Paget has a bone disease named after him - also called Paget's Disease. There is no connection between these diseases, except for the fact that they were both first described by Sir James Paget.

Paget's disease occurs in 1–4% of female patients with breast cancer and may be the only indication of underlying breast cancer. The cancer that it is normally associated with is Ductal Carcinoma In Situ (DCIS).

Men can get Paget's disease but this is extremely rare. It may be associated with Klinefelter's Syndrome, a genetic abnormality that can cause enlarged breasts in men (gynecomastia).

The cause of the symptoms are malignant Paget's cells in the ductal tissue underneath the nipple. The skin (epidermis) becomes involved.

In about half of the cases of Paget's disease there is a lump which indicates that the underlying cancer is more developed.

If there is no lump, the cells are probably limited to the ducts and the skin and the chances of other organs being involved is very small.

Symptoms of Paget's Disease of the Nipple
It is called Paget's Disease of the nipple because that it where the symptoms first appear.

The most common signs are:

• A red, scaly rash on the nipple
• The rash may be itchy or you may notice a burning feeling
• It may spread to the darker area around the nipple (areola)
• The rash may become sores that are weepy - that leak fluid
• It may become crusty due to the discharge
• Your nipple may become inverted
• There may be discharge from the nipple

The symptoms may be diagnosed and treated as a skin rash. If you suspect Paget's look for signs that it is not a common rash.

• Most rashes will spread to both breasts - Paget's does not.
• Most skin rashes will affect the areola and outer skin, Paget's begins at the nipple.

Diagnosing Paget's Disease of the Nipple
The disease is difficult to diagnose. Paget's may be a precancerous condition or it may involve cancer. If it is precancerous, it does not show on many standard tests.

The normal tests for diagnosing Paget's are examining the nipple discharge, mammograms and sonograms and MRIs. The results on the mammograms and sonograms and MRIs will usually come back negative if the underlying cancer is not well developed. This will happen about 50 percent of the time.

• Pathology Exam
  • In many cases, the discharge from the nipple can be examined to see if there are any Paget's cells present.
• Mammogram/Ultrasound/MRI
  • The only sign of Paget's may be a thickening in the area of the nipple and areola. If the cancer is detected, it can be anywhere in the breast. It does not necessarily have to be directly beneath the nipple involved.
• Biopsy
  • If your health care provider suspects Paget's, you will probably need to have a biopsy taken to confirm the diagnosis. This involves taking a small section of skin and breast tissue from the affected area. This is usually done in the doctor's office with a local anesthetic. The tissue is then examined by a pathologist.

One source states: "The neoplastic [cells that have begun to exhibit abnormal behavior] are most numerous in the basal [lower] portion of the nipple epidermis [skin]; therefore, a full thickness biopsy of the skin, rather than a shave biopsy, should be used for diagnosis."

Treatments For Paget's Disease of the Breast
Treatments depend on whether there is a diagnosis of cancer. Since approximately 50 percent of the cases of Paget's found are precancerous, they will require less treatment and have a much better prognosis.

• Surgery
  • Whether you will need surgery and what type of surgery your doctor recommends will depend on the underlying cells. If there is a cancer diagnosis, the treatment will be based on the cancer diagnosis - not the Paget's. An early stage cancer will probably involve a lumpectomy, removing the lump and the breast tissue around it. A more advanced cancer may require a mastectomy, removal of the breast, and other treatments.
  • If you don’t have a lump, a wide local excision, removing the tissue that is involved, followed by radiotherapy may be suggested.
  • For invasive cancer it is important to find out whether the cancer has spread to the lymph nodes in the axilla (glands in your armpit). This may mean removal of the lymph nodes or a Sentinel Node Biopsy.
  • If you have any questions on these procedures, you can access articles by typing the information into the search box. This will find all of the information available on this site, the rest of the About network and the Internet on each topic.
• Radiation therapy
  • In many cases, your health care team will suggest that you have radiotherapy to make sure that any local cancer cells that may have escaped the surgeon's scalpel are killed. If there was no underlying cancer detected, this will probably be the only treatment you require after surgery. In some cases, Paget's is treated with radiation alone and no surgery is necessary. If you opt for a mastectomy, you may not need radiation treatments.
• Chemotherapy/Hormonal Therapy
  • These adjuvant treatments may be prescribed by your health care team to treat the cancer that produced the Paget's symptoms. There are many different drug therapies available. These treatments may involve drugs such as tamoxifen, which are meant to prevent the cancer from growing again. You may need more aggressive treatments if the cancer has spread to your lymph nodes or other sites.

Prognosis
The prognosis with Paget's Disease depends on the diagnosis. If Paget's is diagnosed and treated while it is precancerous the survival rate is extremely high. While the cancer underlying the symptoms is in an early stage, the survival rate is around 95 percent, still very high. The more advanced and widespread the cancer is, the less favorable the prognosis is.

If you suspect that you have the condition, speak to your doctor. Remember that it is extremely rare. This works both for and against you. As with any rare cancer, you may have discovered more about the disease through your research than your doctor is aware of. If you feel that you need a mammogram or a biopsy, do not be shy - request the treatments from your health care provider. However, don't be upset if your doctor disagrees with you.

If your doctor has had any experience with this disease, you will probably get the full work up - unless there are strong indications that you really do just have a rash or an infection of some sort.

If your doctor is not familiar with the condition, try to get a second opinion from someone who has treated Paget's.

Keep in mind that Paget's normally indicates either a precancerous condition or a nonaggressive type of cancer that is in the earliest stages.

Februrary 2, 2003

Last updated: October 23, 2006

Also see -> Paget's Disease of the Nipple Research Studies

Elsewhere on the Web:

Paget's disease of the breast
Paget’s Disease of the Nipple: Questions and Answers
Paget's Disease of the Nipple (Breast Cancer)