Paget's Disease of the Breast/Nipple

Causes and Risk Factors

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Paget's Disease of the Breast/Nipple is a type of breast cancer in which the cancer starts in the cells of the skin of the nipple and then gradually spreads to the areola. It is usually associated with an underlying ductal breast cancer or lobular breast cancer.

It causes an itchy, scaly area which is frequently misdiagnosed as fungal infection or eczema, especially in men.

Causes of Paget's Disease of the Nipple/Breast:

The exact cause is not known. But there are two theories -

  • The cancerous cells, called Paget's cells, reach the skin of the nipple by breaking off from a tumor inside the breast and moving through the milk ducts to the surface of the nipple, resulting in Paget's disease of the nipple. This theory is supported by the fact that more than 97 percent of patients with Paget disease also have underlying invasive breast cancer or ductal carcinoma in situ (DCIS).

  • The second theory suggests that the cancerous changes in the cells on the skin start spontaneously with no connection to cancer elsewhere on the breast. This is seen in very few cases of Paget's disease of the nipple/breast.

Risk factors of Paget's Disease of the Nipple/Breast

Risk Factors

  • Age : Paget's disease is uncommon at a young age and is most common in women around 50-55 years. It is basically a perimenopausal or menopausal condition.
  • Sex : It can also occur in men of the same age, but is quite rare. Paget's Disease is the commonest form of Male breast cancers

  • Ethnicity : White women are more likely to develop breast cancer than black or Hispanic women, but the condition is more severe and spreads faster in black women. So, mortality rate is more in black women.

  • History of breast cancer : A history of breast cancer of any type - lobular or ductal breast cancer can increase the risks of getting Paget's disease in either breast.
  • Family History of breast cancer : A family history of breast cancer, especially in close female relatives like the mother or sister can increase the risks of getting Paget's disease.
  • Benign Breast Abnormalities : Benign conditions like Fibrocystic breast Disease or Atypical hyperplasia can also increase the risks.
  • Radiation exposure : Radiotherapy due to other causes like cancer in the chest or neck can increase the risks of Paget's disease of the nipple and breast.
  • Use of HRT : Some research has suggested that the use of hormone replacement therapy during and after menopause can increase the risk of Invasive Lobular Cancer.
  • Inherited Genetic Risks : Defects in one of several genes, especially BRCA1 or BRCA2, can increase the risks of developing all types of breast cancer, icluding Paget's disease. It also increases the risks of ovarian and other cancers. Women with a rare inherited condition called hereditary diffuse gastric cancer syndrome have an increased risk of both stomach (gastric) cancer and invasive lobular carcinoma.
  • Excess weight : Weighing more than is healthy for your age and height increases your risk of breast cancer - especially after menopause and if you gained weight as an adult.
  • Smoking and Alcohol : Both smoking and alcohol can increase the risk of Paget's disease of the breast and nipple.

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