Paget disease of the breast is a form of breast cancer that affects the skin of the nipple.

• The cause of Paget disease of the breast is poorly understood, but it may be related to tumor cells from other sites in the breast traveling through the milk ducts to the nipple.
• Symptoms and signs of Paget disease of the breast can be similar to those of benign skin conditions and can include itching, redness, thickening, or crusting of the skin.
• Diagnosis of Paget disease of the breast is confirmed by a biopsy, a sample of tissue taken for laboratory analysis and diagnosis. The hallmark sign of Paget disease of the breast is the presence of certain malignant cells known as Paget cells, which are visible on microscopic examination of the tissue biopsy.
• Treatment of Paget disease of the breast typically involves surgery (either breast-conserving surgery or mastectomy). Radiation therapy is given following breast-conserving surgery.
• Depending upon the specific characteristics of the tumor, adjuvant therapy consisting of chemotherapy and/or hormonal therapy, may be given.
• The prognosis and survival rate for Paget disease of the breast depend upon the extent of spread of the cancer and the presence or absence of malignant tumors in the same breast.
• The five-year survival for all women in the U.S. who were diagnosed with Paget disease of the breast between 1988 and 2001 was 82.6%.

Paget disease of the breast key points

• Paget disease of the breast is a rare type of cancer involving the skin of the nipple and, usually, the darker circle of skin around it, known as the areola.
• Most of the time, people with Paget disease of the breast also have one or more tumors inside the same breast.
• Paget disease of the breast may be misdiagnosed at first because its early symptoms are similar to those caused by some benign skin conditions.
• The outlook for people diagnosed with Paget disease of the breast depends on a variety of factors, including the presence or absence of invasive cancer in the affected breast and, if invasive cancer is present, whether or not it has spread to nearby lymph nodes.

What is Paget disease of the breast?

Paget disease of the breast (also known as Paget disease of the nipple and mammary Paget disease) is a rare type of cancer involving the skin of the nipple and, usually, the darker circle of skin around it, which is called the areola. Most people with Paget disease of the breast also have one or more tumors inside the same breast. These breast tumors are either ductal carcinoma in situ or invasive breast cancer.

Paget disease of the breast is named after the 19th century British doctor Sir James Paget, who, in 1874, noted a relationship between changes in the nipple and breast cancer. (Several other diseases are named after Sir James Paget, including Paget disease of bone and extramammary Paget disease, which includes Paget disease of the vulva and Paget disease of the penis. These other diseases are not related to Paget disease of the breast. This fact sheet discusses only Paget disease of the breast.)

Malignant cells known as Paget cells are a telltale sign of Paget disease of the breast. These cells are found in the epidermis (surface layer) of the skin of the nipple and the areola. Paget cells often have a large, round appearance under a microscope; they may be found as single cells or as small groups of cells within the epidermis.

Breast and nipple changes can be a sign of breast cancer. Make an appointment with your doctor if you notice anything unusual.

Paget's disease of the breast affects your nipple and usually the skin (areola) surrounding it. It's easy to mistake the signs and symptoms of Paget's disease of the breast for skin irritation (dermatitis) or another noncancerous (benign) skin condition.

Possible signs and symptoms of Paget's disease of the breast include:

• Flaky or scaly skin on your nipple
• Crusty, oozing or hardened skin resembling eczema on the nipple, areola or both
• Itching
• Redness
• A tingling or Burning sensation
• Straw-colored or bloody nipple discharge
• A flattened or turned-in (inverted) nipple
• A lump in the breast
• Thickening skin on the breast

Signs and symptoms usually occur in one breast only. The disease typically starts in the nipple and may spread to the areola and other areas of the breast.

The skin changes may come and go early on or respond to topical treatment, making it appear as if your skin is healing. On average, signs and symptoms occur for several months before a diagnosis is made.

Doctors don't know what causes Paget's disease of the breast. The most widely accepted theory is that the disease results from an underlying ductal breast cancer. The cancer cells from the original tumor then travel through milk ducts to the nipple and its surrounding skin. Another theory is that the disease can develop independently in the nipple.

For many years, mastectomy, with or without the removal of lymph nodes under the arm on the same side of chest (known as axillary lymph node dissection), was regarded as the standard surgery for Paget disease of the breast. This type of surgery was done because patients with Paget disease of the breast were almost always found to have one or more tumors inside the same breast. Even if only one tumor was present, that tumor could be located several centimeters away from the nipple and areola and would not be removed by surgery on the nipple and areola alone.

Studies have shown, however, that breast-conserving surgery that includes removal of the nipple and areola, followed by whole-breast radiation therapy, is a safe option for people with Paget disease of the breast who do not have a palpable lump in their breast and whose mammograms do not reveal a tumor.

People with Paget disease of the breast who have a breast tumor and are having a mastectomy should be offered sentinel lymph node biopsy to see whether the cancer has spread to the axillary lymph nodes. If cancer cells are found in the sentinel lymph node(s), more extensive axillary lymph node surgery may be needed. Depending on the stage and other features of the underlying breast tumor (for example, the presence or absence of lymph node involvement, estrogen and progesterone receptors in the tumor cells, and HER2 protein overexpression in the tumor cells), adjuvant therapy, consisting of chemotherapy and/or hormonal therapy, may also be recommended.

Risk factors that affect your likelihood of developing Paget's disease of the breast are the same factors that affect your risk of developing any other type of breast cancer.

Some factors that make you more susceptible to breast cancer include:

• Increasing age. Your risk of breast cancer increases as you age.
• A personal history of breast conditions. If you've had a breast biopsy that found lobular carcinoma in situ (LCIS) or atypical hyperplasia of the breast, you have an increased risk of breast cancer.
• A personal history of breast cancer. If you've had breast cancer in one breast, you have an increased risk of developing cancer in the other breast.
• A family history of breast cancer. If your parent, sibling or child was diagnosed with breast cancer, particularly at a young age, your risk of breast cancer is increased. Still, the majority of people diagnosed with breast cancer have no family history of the disease.
• Inherited genes that increase cancer risk. Certain gene mutations that increase the risk of breast cancer can be passed from parents to children. The most well-known gene mutations are referred to as BRCA1 and BRCA2. These genes can greatly increase your risk of breast cancer and other cancers, but they don't make cancer inevitable.
• Radiation exposure. If you received radiation treatments to your chest as a child or young adult, your risk of breast cancer is increased.
• Obesity. Being obese increases your risk of breast cancer.
• Beginning your period at a younger age. Beginning your period before age 12 increases your risk of breast cancer.
• Beginning menopause at an older age. If you began menopause at an older age, you're more likely to develop breast cancer.
• Having your first child at an older age. If you give birth to your first child after age 30, you may have an increased risk of breast cancer.
• Having never been pregnant. If you've never been pregnant, your risk of breast cancer may be increased.
• Postmenopausal hormone therapy. Hormone therapy medications that combine estrogen and progesterone to treat the signs and symptoms of menopause may increase the risk of breast cancer. The risk decreases when you stop taking these medications.
• Drinking alcohol. Drinking alcohol increases the risk of breast cancer.

Paget disease of the breast, also known as Paget's disease of the nipple, is a rare type of breast cancer involving the skin of the nipple and the areola (pigmented area on the breast around the nipple). Paget disease usually affects only one breast.

1. The skin of the nipple and areola often looks crusted, scaly, and red. There may be blood or yellow fluid coming out of the nipple.
2. Sometimes, the nipple looks flat or inverted and might also burn or itch.
The doctor might try to treat this as eczema first, and if it does not improve, recommend a biopsy.

Treatment
1. Paget disease of the breast can be treated by removing the entire breast (mastectomy) or Breast-Conserving Surgery (BCS) followed by whole-breast radiation therapy.
2. If BCS is done, the entire nipple, and areola area also need to be removed. If invasive cancer is found, the lymph nodes under the arm will be checked for cancer.
3. If no lump is felt in the breast tissue, and your biopsy results show cancer has not spread within the breast tissue, the outlook (prognosis) is excellent.
4. If cancer has spread within the breast tissue (is invasive), the outlook is not as good, and cancer will be staged and treated like any other invasive ductal carcinoma.

Symptoms
Itching, tingling, or redness in the nipple and/or areola,Flaking, crusty, or thickened skin on or around the nipple,A flattened nipple,Discharge from the nipple that may be yellowish or bloody
Conditions
A red, scaly rash on the skin of the nipple and areola,Initial findings often include itching (pruritus), scaling, and crusting of and/or discharge from the nipple
Drugs
Surgical removal of breast tissue, adjacent lymph nodes, underlying muscles of the upper chest wall and other tissues (modified radical or radical mastectomy)