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Paget disease of the breast

Paget disease of the breast (Paget disease of the nipple, mammary Paget disease) is a rare type of breast cancer – it involves the skin of the nipple and areola (darker circle of skin around it). According to health experts, Paget’s disease occurs in menopausal women but can also occur in younger women. Most women with Paget disease of the breast also have malignant tumor inside the same breast – often ductal carcinoma in situ or invasive breast cancer. It is an uncommon disease, accounting for 1–4.3% of all the breast carcinomas.

Paget breast cancer was named after British doctor Sir James Paget (XIX century) – he described this malignant disease in 1874; he first reported on the relationship between changes in the nipple-areola region and breast cancer. Paget disease of the breast appears in the nipple-areola zone. Cancerous cells (so called “Paget cells”) are typical sign of Paget disease of the breast. These cells are usually found in the surface layer of the skin (epidermis) of the nipple and the areola. Paget cells have very specific microscopic characteristics.

Paget cancer can occur in both men and women but usually men experience more aggressive flow of the disease due to the smaller size of the mammary glands and easier penetration of cancer cells in the lymph nodes.

Paget disease of the breast – symptoms

Paget disease of the breast

  • Skin changes on one side nipple (scaling, flaking, thickening);
  • Nipple flattening and redness,
  • Unusual nipple discharge (yellowish or bloody or straw-colored);
  • Eczema on the nipple and/or areola;
  • Nipple itching, tingling with burning sensations and pain;
  • Inverted nipple (flattened or turned-in);
  • Thickening skin of the breast;
  • Increased nipple sensitivity;
  • Lump in the breast.

Paget disease of the breast – causes

Unfortunately the cause of this disease is not fully understood. The most popular theory explains the appearance of this malignant disease by “travelling” of cancerous cells from breast tumor through the milk ducts to the nipple and areola. This theory explains why nipple transformations and breast tumor inside the same breast are almost always found together.

According to second theory, the cells in the nipple or areola become cancerous on their own. Scientists suggest that in those cases, nipple skin cells may spontaneously transform into cancer cells. This theory explains cases when nipple cancerous transformations occur independently of breast tumors – in cases on Paget disease without any tumors inside the breast.

Paget disease of the breast – diagnosis

In most cases the diagnosis is based on clinical and cytological data, results of mammography, breast ultrasound, MRI and biopsy.

During Paget breast cancer in 50% of cases changes affect only the nipple-areola zone. In 40% of cases areola-nipple changes combined with palpable tumor in the peripapillary area (surrounding nipple). In 10% cases disease is diagnosed based on microscopic analysis of nipple discharge.

Paget disease of the breast – treatment

Treatment usually include mastectomy followed by radiotherapy, chemotherapy and hormone therapy.

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