Paget's Disease of the Nipple/Breast


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Treatment of Paget's disease is usually successful since spread to distant organs is slow. Surgery can help in total removal of the cancer cells. There are different treatment procedures depending on the size of the tumor, on whether there is an underlying invasive cancer and whether there is spread to surrounding tissues and lymph nodes.

The first step in management is a biopsy of the affected skin to diagnose paget's disease. The presence of Paget's cells ( a typical large cell with clear cytoplasm found in this disease) confirms the diagnosis.

A mammogram should then be done to identify the presence of underlying breast cancers like Invasive Ductal Cancer (IDC). IDC is present in more than 90% of all cases of paget's disease.

If no lump or mass is seen on mammogram, an MRI is done to confirm the absence of a mass. If the MRI rules out the presence of any breast cancer, then surgical excision of the affected areolar/nipple complex is the standard treatment. Follow up treatment will depend on the pathology of the the removed tissue.

If the initial mammogram or MRI shows a breast lump suggestive of IDC or ILC, then treatment is focussed on treating the breast cancer as well as the skin changes. Surgery is the standard treatment followed by adjuvent treatment procedures like radiotherapy, chemotherapy or hormone therapy.

Treatment Plan of Paget's Disease of the Nipple/Breast

Treatment options include:


  • Lumpectomy : For many years, mastectomy was the only treatment offered to the patient since patients with Paget disease of the breast were almost always found to have one or more invasive cancers either Invasive Ductal Cancer or Invasive Lobular Cancer, inside the same breast. Even if only one tumor was present, that tumor could be located several centimeters away from the nipple and areola and would not be removed by surgery on the nipple and areola alone.

    But is is now seen that breast conserving surgeries like lumpectomy followed by radiotherapy is usually successful in treatment of Paget's disease. Survival rates are also the same. A breast-conserving surgery consists in the removal of the nipple, areola and the part of the breast that is affected by cancer. The goal is to take out the cancer and a small margin of healthy tissue around it, just to be sure that no cancer is left behind.

  • Mastectomy : A mastectomy with removal of the entire breast may be necessary if there is underlying invasive cancer.

    It is also done when no clear margin or healthy tissue is obtained after a lumpectomy or if the woman is unable to undergo radiotherapy after lumpectomy.

    Dissection of the lymph nodes may also be necessary in some cases where there is spread of the disease to the lymph nodes.

    Adjuvent Therapy : Adjuvent therapy with either radiotherapy or chemotherapy may be needed to prevent recurrence of the cancer. Radiotherapy is the preferred treatment in cases where the rash is limited to the breast.

    Hormonal therapy may also be necessary in some women. Hormonal therapy reduces the production of hormones within the body, or prevents the hormones from stimulating the cancer cells to grow

    Prognosis of Paget's Disease:

    The prognosis for people with Paget's disease of the breast depends on a variety of factors:

    The 5-year relative survival for all women in the U.S. diagnosed with this condition from 1988 to 2001 was 82.6%. This compares with a 5-year relative survival of 87.1% for women diagnosed with any type of breast cancer in that same time.

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