What Is Paget's Disease of the Nipple?

This rare type of breast cancer presents with changes in the nipple or areola

Breast cancer, conceptual image
Paget's Disease. IAN HOOTON / Getty Images

Paget’s disease of the nipple or breast is a rare type of breast cancer which can occur in women and men. It involves the nipple and the darker skin around it, called the areola, and it usually signals the presence of another breast cancer deeper within the same breast. These breast tumors may be ductal carcinoma in situ or invasive breast cancer. Symptoms often include a scaly, red, itchy nipple and areola.

How Common is Paget's Disease of the Nipple? 

Only 1 to 4 percent of all cases of breast cancer include Paget's disease of the breast, and most cases are found in menopausal women, but can also appear in adolescents and the elderly. 

The disease is named after Sir James Paget, a British doctor from the 1800s who published articles about the link between changes in the nipple and underlying breast cancer.  

 

Is There Always Another Underlying Cancer? 

In a few cases of Paget’s disease, there is no underlying breast cancer, or if a tumor is present, it is unrelated to the disease in the nipple. Researchers suggest that in those cases, nipple skin cells may spontaneously change into cancer cells.

However, the majority of people with Paget's disease of the nipple have either invasive breast cancer or ductal carcinoma in situ (DCIS). Researchers believe that cancer cells may travel from the tumor and float up through the milk ducts, where they enter the nipple and areola.

 Breast tumors are almost always found in the same breast that has Paget disease. 

Signs and Symptoms of Paget's Disease of the Nipple

  • Red, scaly, crusty, and/or flaky nipple and/or areola 
  • Mild irritation of the skin
  • Tingling in nipple skin
  • Itchiness that doesn’t respond to creams
  • Very sensitive skin on the nipple
  • Burning or painful nipple skin
  • Ooze or bloody discharge from the nipple (not milk)
  • Nipple retraction (when it pulls into the breast/flattens)
  • Breast lump beneath the affected skin

Diagnosing Paget's Disease 

If you are experiencing any of the above signs and symptoms, see a health professional. Diagnosing Paget's disease will include the following steps: 

  • Physical examination of the nipple and breast. Up to 50 percent of people with Paget's disease of the nipple have a breast lump that is felt upon clinical exam. 
  • Biopsy and pathology of the nipple or areola, which may consist of one of the following: A surface biopsy (cells are gently scraped from the surface of skin), shave biopsy (top layer of skin is removed), punch biopsy (a disk-shaped piece of tissue is removed with a circular cutting tool), or wedge biopsy (a small wedge of tissue is removed with a scalpel). 
  • Mammography, ultrasound, and/or MRI to get images of the breast and pinpoint areas of concern/possible tumors. 

Treating Paget's Disease

Treatment for Paget's disease depends on the size and stage of cancer. The most common treatments are: 

  • Mastectomy if tumors are found within the breast
  • Sentinel lymph node biopsy, which determines whether or not cancer has spread to lymph nodes
  • Chemotherapy or hormonal therapy, depending on the stage of the tumor and other features of the cancer
  • Breast-conserving surgery, which includes removal of the nipple and areola, followed by whole-breast radiation therapy, if no tumors are found

Sources:

Paget’s Disease of the Nipple: Questions and Answers. Cancer Topics. National Cancer Institute. April 2012. Paget’s Disease of the Nipple: Questions and Answers
Paget's Disease of the Nipple. American Cancer Society. 
Paget Disease of the Breast. NIH: National Cancer Institute. 

Continue Reading