Generality
Nipple Paget's disease, also known as Paget's disease of the breast, is a rare form of breast cancer.
Paget's disease of the breast occurs more often in people over the age of 50 and affects 1-2 in 100 women with breast cancer. The disease can be misdiagnosed at first, as at the onset the symptoms are similar to those caused by some benign skin conditions. Nipple Paget's disease is often associated with other forms of breast cancer and is usually treated in the same way, so surgery is likely to be necessary. The prospects for patients suffering from Paget's disease of the nipple depend on a variety of factors, including the presence or absence of an invasive breast carcinoma involved and the possible spread to nearby lymph nodes. If Paget's disease is detected and treated in its early stages, there is a good chance of recovery.
See: Photo Nipple of Paget of the Nipple
Symptoms
To learn more: Symptoms Paget's disease of the Nipple
Paget's disease affects the skin of a nipple and can extend to the areola (a highly pigmented area surrounding the nipple) or to other areas of the breast. Clinical signs usually involve only one breast.
Symptoms of Paget's disease of the nipple are easily confused with skin irritations (dermatitis or eczema) or with other benign skin conditions (non-cancerous).
Possible signs and symptoms of Paget's disease of the breast include:
- Dry, irritated or scaly skin, with symptoms that are often similar in appearance to those of an eczema on the nipple, on the areola, or both;
- Redness, itching, tingling or burning sensation;
- Nipple secretions of straw color (lymphatic or purulent exudation from the nipple)
- Inversion or introflection of the nipple;
- Breast lump.
Skin changes that involve the nipple can begin and resolve within a short time or respond to topical treatment, making it appear that the skin is on the mend. On average, the patient may experience signs and symptoms limited to a superficial appearance for 6-8 months, before the correct diagnosis is formulated. In reality, the appearance of these inflammatory-like skin alterations is indicative of a very serious underlying condition. To highlight a probable lesion associated with Paget's disease, it may be useful to regularly check the nipple and areola of both breasts during breast self-examination. If a lump or a change in the appearance or shape of a breast is felt, or if itching and irritation appear that last for more than a month, it is advisable to consult a doctor. The sooner a cancerous lesion is diagnosed, the greater the chances of successfully treating the disease.
Causes
Paget's disease is usually a sign of breast cancer that originates in the tissue behind the nipple (ductal carcinoma in situ) or in other parts of the breast (invasive carcinoma). In about half of all cases of Paget's disease, a nodule is found in the breast. However, the exact cause underlying the condition has not yet been understood.
The most accepted theory is that cancer cells derive from breast cancer and migrate through the galactophore ducts invading the surrounding breast tissue, including the nipple and areola. This would explain why Paget's disease of the breast and ductal breast cancer within the same breast are almost always associated.
A second theory is that the cells in the nipple and in the areola become cancerous independently. This would explain why some patients develop the malignant condition without having a tumor inside the same breast. Furthermore, it is possible that Paget's disease of the nipple and tumors within the same breast may develop due to the outcome of two distinct neoplastic processes.
The risk factors that influence the likelihood of developing nipple Paget's disease are the same as those that predispose to the development of other types of breast cancer.
Diagnosis
A nipple biopsy allows doctors to correctly diagnose Paget's disease. This exam consists of scraping superficial skin cells or removing a small tissue sample for microscopic analysis. A pathologist examines the collected cells or tissues under the microscope, looking for particular malignant cells, also known as Paget cells . These can be found as single cells or small clusters of tumor clones within the nipple epidermis and areola, and represent a telltale sign of the pathology.
Most people with Paget's disease also have one or more cancers within the same breast. In addition to the nipple biopsy, the doctor must perform a clinical breast examination to physically check for unusual areas. During this examination, the doctor checks the appearance of the skin around the nipples and the possible presence of nodules, areas of thickening or other changes: 50% of people with Paget's disease of the nipple have a lump that can be detected during of a clinical breast examination.
The doctor may order further investigations to confirm the diagnosis, such as:
- Mammography : it is an X-ray examination of breast tissue, which can indicate whether nipple changes are associated with breast cancer. If the results of mammography in both breasts do not reveal signs of cancer, the doctor can perform an ultrasound scan or an MRI scan, to look for any tumors that cannot be detected with mammography.
- MRI : it is often used to assess the extent of breast cancer. The results can help determine which surgical procedure should be performed.
- Sentinel lymph node biopsy : in the case of invasive breast cancer, the sentinel lymph nodes must be examined under the arm (axillary lymph nodes), the first to be metastasized in the presence of malignant tumors. During the procedure, the surgeon identifies a lymph node and removes it to check if the cancer cells have spread to this area.
Nipple Paget's disease may be "misdiagnosed" at first, because the disease is rare and symptoms may suggest a benign condition of the skin. Patients often present symptoms for several months before the condition is properly diagnosed.
Treatment
For many years, mastectomy - with or without removal of axillary lymph nodes on the same side of the chest (procedure known as lymphadenectomy ) - has been considered the standard surgical approach for Paget's nipple disease. This type of intervention is justified by the fact that in many cases, together with paget's disease, there is also a tumor inside the same breast, which could be located several centimeters from the nipple and from the areola.
Subsequently, studies have shown that breast-conserving surgery, which involves removing the nipple, areola and a part of the breast affected by cancer, followed by radiotherapy, is a safe choice for patients who do not have a palpable nodule in the breast and whose mammograms do not reveal a tumor.
People with Paget's disease of the nipple who have breast cancer and are undergoing a mastectomy should undergo a sentinel node biopsy to assess whether the cancer has spread to the axillary lymph nodes. If the tumor cells are in the sentinel node, a more extensive surgical procedure may be necessary. Depending on the stage of development and other characteristics of breast cancer, adjuvant therapy may also be recommended, consisting of chemotherapy, radiotherapy and / or hormone therapy .
To learn more: Drugs for the treatment of Paget's disease of the Nipple »
The prognosis for people with Paget's nipple disease depends on a variety of factors. The presence of an invasive carcinoma in the affected breast and the spread of cancer to neighboring lymph nodes are associated with reduced survival.
Adjuvant therapy. After surgery, your doctor may recommend additional treatment (adjuvant therapy) with chemotherapy drugs, radiotherapy or hormone therapy to prevent a recurrence of breast cancer and to destroy any remaining cancer cells. The specific treatment depends on the degree of Paget's disease of the nipple and on the positivity or otherwise of tumor tests for some characteristics, such as the presence of a lymph node involvement, or the expression in cancer cells of estrogen and progesterone receptors or of the HER2 protein.
