Paget’s Disease of the Breast

Paget's disease of the breast is a rare form of breast cancer that affects the nipple and the dark skin around it (areola). It often looks like a skin condition, such as eczema or psoriasis, but it is caused by cancer cells. In most cases, it is linked to an underlying breast cancer within the breast tissue itself, which can be either non-invasive or invasive.

What is Paget's Disease of the Breast?

Paget's disease of the breast is an uncommon type of breast cancer that starts in the milk ducts and spreads to the skin of the nipple and areola. It is rare, accounting for about 1 to 4 out of every 100 breast cancers (1-4%). This condition almost always indicates the presence of an underlying breast cancer within the same breast.

Paget's disease of the breast is distinct because it primarily affects the skin of the nipple and the surrounding darker area (areola). The cancer cells, called Paget cells, are found in the outer layer of the skin (epidermis) of these areas. While it appears on the surface, Paget's disease of the breast is usually a sign of a deeper issue. In most cases, it is associated with an underlying breast cancer. This underlying cancer can be either ductal carcinoma in situ (DCIS), which is non-invasive and confined to the milk ducts, or an invasive breast cancer, which has spread beyond the ducts into surrounding breast tissue. Because of its appearance, Paget's disease can often be mistaken for common skin conditions like eczema or psoriasis. However, unlike these conditions, Paget's disease does not typically respond to standard topical treatments and usually affects only one breast. Early recognition is important for effective treatment.

Symptoms

The symptoms of Paget's disease of the breast typically appear on the nipple and areola, usually affecting only one breast. These changes often resemble a skin irritation and can include redness, scaling, crusting, itching, or a burning sensation. It's important to note that these symptoms usually persist and do not improve with typical skin creams.

The initial signs of Paget's disease of the breast often involve changes to the nipple and areola that can be subtle. You might notice the skin becoming flaky, scaly, or crusty. This can be accompanied by persistent itching, tingling, or a burning sensation in the affected area. Many people initially mistake these symptoms for a common skin condition like eczema or psoriasis. As the disease progresses, the nipple itself may undergo changes. It might become flattened or even turn inward (inverted). Some people also experience a yellowish or bloody discharge from the nipple. In some cases, a lump may be felt in the breast, which indicates a more advanced underlying breast cancer. It's crucial to remember that these symptoms typically affect only one breast. If you have persistent skin changes on your nipple or areola that do not get better with over-the-counter creams or other treatments, it is important to see a doctor for evaluation.

Causes & risk factors

Paget's disease of the breast occurs when cancer cells from an existing tumor inside the breast travel through the milk ducts to the nipple and areola. The exact cause of this cell migration isn't fully understood, but the risk factors are generally the same as those for other types of breast cancer, including age and family history.

The most widely accepted theory for the development of Paget's disease of the breast is that cancer cells originate from an underlying breast tumor, usually a ductal carcinoma, and then migrate along the milk ducts to the nipple and areola. This explains why an underlying breast cancer is almost always present when Paget's disease is diagnosed. Less commonly, it's thought that the cells in the nipple itself may become cancerous independently. Because Paget's disease of the breast is a form of breast cancer, its risk factors are similar to those for other types of breast cancer. These include increasing age, a personal or family history of breast cancer, and certain genetic mutations, such as those in the BRCA1 or BRCA2 genes. Other factors like obesity, alcohol consumption, radiation exposure to the chest, and certain types of hormone therapy can also increase risk. It's important to understand that having one or more risk factors does not mean you will definitely develop Paget's disease or any other type of breast cancer. Many people with risk factors never develop the disease, and some people with no known risk factors do. Regular screenings and prompt evaluation of any new symptoms are key.

How it's diagnosed

Diagnosing Paget's disease of the breast typically involves a physical exam, imaging tests, and a biopsy. A doctor will examine the nipple and areola, and imaging tests like a mammogram, ultrasound, or MRI are used to look for an underlying tumor. A biopsy, which involves taking a small tissue sample, is essential to confirm the diagnosis.

The diagnostic process usually begins with a thorough physical examination of your breast, nipple, and areola by a healthcare provider. They will look for the characteristic skin changes and feel for any lumps in the breast. Because Paget's disease can mimic other skin conditions, a high index of suspicion is often needed. Imaging tests are crucial for identifying any underlying breast cancer. A mammogram is typically the first imaging test. Depending on the findings, your doctor may also recommend an ultrasound of the breast or a magnetic resonance imaging (MRI) scan. These tests help to determine the extent of any underlying tumor and whether it is invasive or non-invasive. The definitive diagnosis of Paget's disease of the breast requires a biopsy. This involves taking a small tissue sample from the affected nipple or areola skin (a punch biopsy). If a lump is present in the breast, a core needle biopsy will also be performed. The tissue samples are then examined under a microscope by a pathologist to identify Paget cells and confirm the presence and type of cancer.

Treatment options

Treatment for Paget's disease of the breast primarily involves surgery to remove the affected nipple and areola, and any underlying breast cancer. The type of surgery depends on the extent of the disease. Additional treatments like radiation therapy, chemotherapy, hormone therapy, or targeted therapy may be recommended based on the characteristics of the underlying cancer.

The main treatment for Paget's disease of the breast is surgery. If the underlying cancer is extensive or invasive, or if you prefer, a mastectomy (removal of the entire breast) may be performed. If the underlying cancer is small or only involves the nipple and areola without an invasive component, breast-conserving surgery (lumpectomy), which removes the nipple, areola, and a margin of healthy tissue, may be an option. Breast-conserving surgery is usually followed by radiation therapy to the remaining breast tissue to reduce the risk of recurrence. After surgery, additional treatments, known as systemic therapies, may be recommended. These treatments target cancer cells throughout the body. The choice of systemic therapy depends heavily on the characteristics of the underlying breast cancer, such as its stage, whether it's invasive, and its hormone receptor status (estrogen receptor-positive, progesterone receptor-positive) or HER2 status. Systemic therapies can include chemotherapy, which uses drugs to kill cancer cells; hormone therapy, which blocks hormones that fuel certain breast cancers; and targeted therapy, which focuses on specific weaknesses in cancer cells. Your treatment plan will be personalized by your healthcare team based on the specific details of your diagnosis to offer the most effective care.

Recovery & outlook

The recovery and outlook for Paget's disease of the breast largely depend on whether an underlying invasive breast cancer is present and its stage. If the disease is confined to the nipple and areola without an invasive underlying tumor, the prognosis is generally very good. With invasive cancer, the outlook depends on how early it was detected and its specific characteristics.

Recovery from Paget's disease of the breast involves healing from surgery and managing any side effects from additional treatments like radiation or chemotherapy. Your healthcare team will provide guidance on wound care, pain management, and rehabilitation exercises, especially after mastectomy or lymph node removal. Regular follow-up appointments are crucial to monitor your recovery and check for any signs of recurrence. The outlook for people with Paget's disease of the breast varies significantly. If the disease is diagnosed early and there is no underlying invasive breast cancer (meaning it's only ductal carcinoma in situ or confined to the nipple epidermis), the prognosis is excellent, with very high survival rates. This highlights the importance of early detection and diagnosis. However, if an underlying invasive breast cancer is present, the outlook depends on the stage of that cancer, including its size, whether it has spread to lymph nodes, and its biological characteristics. In these cases, the prognosis is similar to that of other invasive breast cancers of the same stage. Your medical team will discuss your specific prognosis based on all these factors, offering the most accurate information for your situation.

When to see a doctor

You should see a doctor if you notice any persistent changes to your nipple or the dark skin around it (areola), especially if these changes do not improve with over-the-counter creams or other home remedies. Symptoms like redness, scaling, crusting, itching, burning, or nipple discharge, particularly if they affect only one breast, warrant medical evaluation.

It is important to seek medical attention promptly if you experience any new or persistent skin changes on your nipple or areola. Do not assume these symptoms are just a minor skin irritation like eczema, especially if they do not respond to typical treatments within a few weeks. Early diagnosis of Paget's disease of the breast can significantly improve treatment outcomes. Specific signs that should prompt a visit to your doctor include a nipple that becomes flattened or inverted, any yellowish or bloody discharge from the nipple, or a lump that you can feel in your breast. While these symptoms can be caused by many conditions, it is crucial to rule out Paget's disease of the breast and any associated underlying cancer. Your doctor will be able to perform a physical examination and recommend appropriate diagnostic tests, such as mammography or a biopsy, to determine the cause of your symptoms. Being proactive about any unusual breast changes is a key step in maintaining your breast health.

Frequently asked questions

Is Paget's disease of the breast always cancer?

Yes, Paget's disease of the breast is always considered a form of cancer. It involves the presence of cancerous cells (Paget cells) in the skin of the nipple and areola. In most cases, it is also associated with an underlying breast cancer within the breast tissue itself, which can be either non-invasive or invasive.

Can Paget's disease of the breast spread?

Paget's disease of the breast itself is a manifestation of cancer on the nipple and areola. The underlying breast cancer, which is almost always present, can spread. If the underlying cancer is invasive, it has the potential to spread to nearby lymph nodes and other parts of the body, similar to other types of invasive breast cancer.

Is Paget's disease of the breast painful?

Pain is not always a primary symptom of Paget's disease of the breast, but some people do experience discomfort. Symptoms often include itching, burning, or tingling sensations, which can be irritating or painful. Any pain or tenderness in the breast or nipple area should be discussed with a doctor.

How rare is Paget's disease of the breast?

Paget's disease of the breast is considered rare, accounting for a small percentage of all breast cancer diagnoses. It is estimated to occur in about 1 to 4 out of every 100 cases of breast cancer (1-4%).

Can men get Paget's disease of the breast?

Yes, men can also develop Paget's disease of the breast, although it is extremely rare. Like in women, it is almost always associated with an underlying breast cancer. The symptoms and diagnostic process are similar for both men and women.

Does Paget's disease of the breast look like eczema?

Yes, Paget's disease of the breast often looks very similar to eczema or other common skin conditions. It can cause redness, scaling, crusting, and itching on the nipple and areola. However, unlike eczema, Paget's disease typically affects only one breast and does not respond to standard eczema treatments.

Sources

• MedlinePlus — Paget's Disease of the Breast
• Mayo Clinic — Paget's Disease of the Breast
• Cochrane Library — Paget's Disease of the Breast