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Paget's Disease of the Nipple

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Paget's Disease of the Nipple

The first noticeable signs of breast cancer are usually lumps or abnormalities found beneath the surface of the breast during a physical examination or on the x-ray imagery of a screening mammogram. There is one type of breast cancer, however, that first shows its presence right on the surface of the breast, in the area of the nipple and the surrounding areola. This condition is called Paget's disease of the nipple.

Paget's disease is in itself a form of breast cancer, however, in about 97% of all cases that are diagnosed, another kind of breast cancer is also eventually discovered in underlying tissue, inside the lining of the milk ducts of the breast. This type of cancer will be either invasive ductal carcinoma, or the non-invasive ductal carcinoma in situ; but regardless of whether or not it spreads to other parts of the body, ductal carcinomas can apparently trigger attacks against the nipple in a small number of instances. Overall, less than 5% of breast cancer patients are diagnosed with Paget's disease of the nipple.

The Symptoms
One of the problems with Paget's disease is that its symptoms to some extent mimic dermatitis and other benign conditions, at least in its early stages. Consequently, the average time between the appearance of Paget's disease and its actual diagnosis by a physician is between six and eight months, as most women simply do not realize something is seriously wrong when this type of breast cancer first develops.

The most common symptoms of Paget's disease of the nipple include:

  • Redness of the nipple and the areola
  • Scaly or flaky skin surrounding the nipple
  • Persistent itching
  • A burning sensation
  • Oozing and crusting of the skin
  • Bloody or brown nipple discharge
  • Thickening of the surface of the areola
  • The appearance of detectable lumps in the breast just below the nipple

While Paget's disease can strike younger women, the majority of those who develop these symptoms and receive a diagnosis are past the age of 50.

How it Develops There are two theories about how Paget's disease may develop. The first is that cancerous cells may be able to break off or separate from underlying ductal tumors and migrate to the surface of the breast, where they can then infect the nipple and areola. Because 97% of all Paget's disease sufferers will eventually be diagnosed with one or another type of ductal breast cancer, this theory obviously makes sense. The fact that a small subclass of Paget's victims do not have any other kind of breast cancer contradicts this idea, however, and this has led to speculation that at least in some cases tissue cells in the nipple may spontaneously mutate into cancer cells, just as they do with any other kind of breast cancer tumors.

These two possibilities are not mutually exclusive, and it is possible that each may be valid in some cases.

Diagnosis and Treatment
The only sure way to detect Paget's disease of the nipple is to take tissue samples from the area for a biopsy. If a woman tests positive for cancer, the next step will be a diagnostic mammogram combined with a clinical examination to see if another type of breast cancer is present in the underlying tissue – and as already mentioned, in most cases such a cancer will be found.

Only if invasive ductal breast cancer is diagnosed and has begun the process of metastasis (spreading) will it be necessary to perform a full mastectomy on a woman suffering from Paget's disease. If the carcinoma inside the breast is of the non-invasive variety, then a lumpectomy to remove the nipple, areola, and the deeper breast cancer tumor will be sufficient, and as is usually the case this treatment will be combined with radiation therapy designed to kill any remaining cancer cells in the breast. Naturally, if invasive breast cancer is found to have spread beyond the breast to other areas of the body, additional treatment for cancer such as chemotherapy may be required.

Raising Awareness
Because women do not usually recognize Paget's disease for what it is, many delay seeking medical attention, and the result is that any underlying invasive cancer that might be present will have more time to metastasize and migrate beyond the milk ducts of the breast. For this reason, promoting greater awareness about Paget's disease is critically important. Whenever a woman is experiencing any of the symptoms associated with this type of cancer, she should make an appointment to see a doctor as soon as possible. The fact that this is not happening frequently enough now shows that medical professionals still have a lot of work to do to spread the word about Paget's disease of the nipple.

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