Paget Disease of the Breast (Paget's Disease of the Nipple)
The prognosis of Paget’s disease of the breast depends on the type and stage of underlying cancer. The five-year survival rate for early diagnosis is 95.8 percent.

Paget’s disease of the breast (mammary Paget’s disease) is a rare form of cancer that usually involves the nipple and darker region around it (called the areola). It is usually associated with underlying breast cancer that may be either ductal carcinoma in situ or invasive breast carcinoma. Paget’s disease of the breast is not related to Paget’s disease of the bone.

Paget’s disease of the breast is treatable, especially when diagnosed at an early stage. The prognosis (survival rate) depends on various factors, such as the type of underlying cancer (ductal carcinoma in situ or invasive breast carcinoma) and stage of cancer (in case of invasive breast cancer). Depending on the stage of invasive breast cancer, five-year survival rates (percentage of people with Paget’s disease of the breast who will live for at least five years after diagnosis) for women with Paget’s disease and invasive cancer in the same breast can be seen below.

Table. The five-year survival rate for Paget's disease of the breast by stage
Stage of cancer Five-year survival rate percentage (%)
Stage I 95.8%
Stage II 77.7%
Stage III 46.3%
Stage IV 14.3%

What are the risk factors for Paget’s disease of the breast?

Common risk factors of Paget’s disease of the breast include:

  • Age: Risk increases with increasing age.
  • Sex: More common in women than in men.
  • Weight: Obesity and dense breast tissue have more predominance.
  • Family history: Having family members with the same disease increases the risk of Paget’s disease.
  • Cancer history: Women having a positive cancer history (specifically breast cancer) have more risk of Paget’s disease.
  • Alcohol: Heavy drinkers are more susceptible.
  • Radiation exposure: People undergoing radiation treatment on their chest are more prone to Paget’s disease.
  • Hormone replacement therapy: Greater exposure to estrogen and progesterone increases the risk of malignancy.

What are the signs and symptoms of Paget’s disease of the breast?

Paget's disease of the breast always starts in the nipple and may extend to the areola, appearing as a red, scaly rash. Additionally, the following symptoms of the disease are commonly seen around the nipple region, including:

  • Pain
  • Redness
  • Itching
  • Burning sensation
  • Discharge
  • Tingling
  • Inverted nipple
  • Lump
  • Bleeding
  • Thickened skin

These symptoms are commonly mistaken for some inflammatory conditions.

However, if these are present persistently for a very long time, then you should get them checked by a specialist.

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Skin Cancer Symptoms, Types, Images See Slideshow

How is the diagnosis of Paget’s disease of the breast made?

The following methods are commonly used to make the diagnosis.

Biopsy

A small piece of tissue is cut, and cells are visualized under the microscope to see if there is any malignant change.

  • Surface biopsy: Cells are taken from the surface of the breasts.
  • Shave biopsy: A sharp tool like a shave is used to take the top layer of the skin.
  • Punch biopsy: A circular cutting tool is used that punches out a little of the affected breast tissue.
  • Wedge biopsy: A wedge of the tissue is collected for lab examination.

Imaging methods

What are the complications of Paget’s disease of the breast?

Complications of Paget’s disease of the breast include:

How can Paget’s disease of the breast be prevented?

Although there is no sure way to prevent Paget’s disease of the breast, certain measures may lower your risk, such as:

  • Maintenance of healthy body weight
  • Regular exercise
  • Limitations of alcohol and avoiding smoking
  • Healthy diet

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Medically Reviewed on 9/22/2021
References
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3263015/

https://emedicine.medscape.com/article/1101235-overview

https://www.cancer.gov/types/breast/paget-breast-fact-sheet