Understanding Rare Paget's Disease of the Breast Cancer Symptoms

Understanding Paget's Disease of the Breast

Paget's disease of the breast is a rare type of breast cancer involving the skin of the nipple. While the condition only accounts for about 1-4% of all breast cancers, being informed about the symptoms, diagnosis, and treatment is important.

The characteristic skin changes of Paget’s disease can help identify the condition early on. With proper treatment, many patients go on to have a favorable outlook.

What is Paget’s Disease of the Breast?

Paget’s disease of the breast refers to a condition where cancer cells originate in the milk ducts of the nipple and then spread to the surface of the nipple skin.

This leads to visible skin changes on or around the nipple. There is often an underlying breast cancer present as well, such as ductal carcinoma in situ (DCIS) or invasive breast cancer.

The disease was first described by British surgeon Sir James Paget in 1874. It typically comprises just 1-4% of all breast cancers.

Symptoms of Paget’s Disease

The main symptom of Paget’s disease is change to the appearance of the nipple skin. Signs can include:

Redness, irritation, or a scaly rash affecting the nipple skin
Itching or tingling sensation in the nipple
Flattening or indentation of the nipple
Oozing, bleeding, or discharge from the nipple
Burning or pain in the nipple area

These skin changes are caused by the underlying breast cancer cells accumulating within the milk ducts and spreading into the nipple tissue.

In about 50-60% of cases, patients with Paget’s disease will also have a lump or mass in the breast upon examination. Nipple discharge may also be noted in some cases.

Causes and Risk Factors

The exact cause of Paget’s disease is unknown, but the condition always relates to underlying breast cancer.

Cancerous cells originating in the milk ducts travel up and extend into the nipple epidermis, causing the characteristic skin changes.

Factors that may increase the risk for Paget’s disease include:

Older age (more common after menopause)
Family history of breast cancer
Inherited gene mutations like BRCA1/BRCA2
Radiation exposure to the chest
High breast tissue density
Not having children or having them later in life

There are often no identifiable risk factors, however. Paget’s disease can occur in both men and women, but is much more prevalent in females.

How is Paget’s Disease Diagnosed?

Paget’s disease is diagnosed based on the characteristic appearance and feel of the nipple skin, along with breast imaging and biopsy.

Your doctor will first perform a clinical breast exam looking for any skin changes, masses, or discharge. Breast imaging tests may include:

Mammogram: X-ray imaging of the breast tissue.
Breast ultrasound: Uses soundwaves to evaluate masses or abnormalities.
MRI: Detailed breast imaging using magnetic fields and radio waves.
PET scan: Looks for areas of increased metabolic activity.

These tests help identify any underlying breast tumors that may be present.

To confirm Paget’s disease, your doctor will take a small sample of tissue from the affected nipple area to examine under a microscope. This biopsy can determine if cancer cells are present.

Paget’s Disease Staging

If Paget’s disease is diagnosed, additional testing helps determine how far the cancer has spread. This process is called staging.

Stage 0 means the cancer is confined to just the nipple skin.

In stage 1, the cancer has spread to the nipple ducts and milk glands.

Stage 2 indicates the tumor has grown into breast tissue, while stage 3 means the cancer has spread to the lymph nodes or chest muscles.

Stage 4 means the cancer has metastasized to distant sites like the lungs, liver or brain.

Staging helps guide the appropriate treatment approach.

Treatment Options for Paget’s Disease

Common treatments for Paget’s disease include:

Lumpectomy: Removing just the tumor and affected breast tissue.
Mastectomy: Removing the entire breast along with the nipple.
Radiation: Using targeted radiation beams to kill cancer cells.
Chemotherapy: Powerful anti-cancer medications that kill rapidly dividing cells.
Hormone therapy: Medications to reduce estrogen production in hormone-positive cancers.

The best treatment depends on the stage, the patient’s age and health status, and whether or not an underlying breast tumor is present.

Many patients undergo a combination of surgery with radiation, chemotherapy, or hormone therapy to ensure the greatest chance of destroying all cancer cells.

Paget's Disease Prognosis and Outlook

The prognosis for Paget’s disease depends on how early it is diagnosed and the stage of the underlying breast cancer.

If Paget’s disease is caught very early when confined to just the nipple, the 5-year survival rate is close to 100%.

Once the cancer spreads to the breast tissue or lymph nodes, the prognosis becomes less favorable. However, survival rates have improved dramatically in recent decades with advances in screening and treatment.

All patients with Paget’s disease should discuss statistics specific to their unique case with their oncology team.

Paget's Disease Pictures and Appearance

Being able to identify the characteristic signs of Paget’s disease on the nipple is crucial for early detection and prompt treatment. Here are pictures reflecting the skin changes that may occur:

Redness and Rash

One of the first signs of Paget’s disease is often redness or a rash affecting the nipple skin and areola complex. The area may look bright red, pink, or irritated.

Scaling

A white, flaky rash resembling eczema is common. The nipple skin may look dry, scaly, and discolored.

Erosion

Over time, the nipple skin can become thinner and eroded. Cracking, oozing, or crusting lesions may be present.

Nipple Flattening

The cancer cells accumulating within the milk ducts can cause the nipple to flatten out or become indented.

Discharge

Some patients note clear or bloody discharge seeping from the nipple. Any nipple discharge should be evaluated promptly.

Enlarged Lymph Nodes

If the breast cancer has spread to the nearby lymph nodes, they may become visibly enlarged or swollen under the arm or around the collarbone.

Underlying Breast Lump

There is often a hard, irregular mass or lump within the breast tissue that can be felt on examination in many cases of Paget’s disease.

Living with Paget's Disease

Being diagnosed with Paget’s disease of the breast is frightening. Many patients feel shocked, anxious, depressed, or angry. Adjusting to life after treatment for Paget’s disease also poses challenges for survivors.

Some tips for coping include:

Learning about the diagnosis and treatment options
Joining a support group to connect with other patients
Seeking counseling or therapy to process emotions
Relying on family and friends for practical support
Focusing on positive progress each day
Practicing self-care through rest, healthy eating, exercise, and relaxing activities
Communicating openly with your healthcare team
Being diligent about follow-up care and monitoring for recurrence

It is normal to feel scared, anxious, angry, or down after being diagnosed with breast cancer. Give yourself permission to experience the emotions. With time, support, and a positive attitude, many survivors are able to adjust and enjoy full lives after Paget’s disease.

Paget’s Disease Support Groups

Online forums allow you to connect with others affected by Paget’s disease and rare breast cancers. You can share experiences, find resources, and receive encouragement from those who understand what you are going through. Here are some options:

Know that you are not alone. Connect with others dealing with this rare breast cancer for empowerment and hope.

Importance of Paget's Disease Awareness

Because Paget’s disease of the breast is rare, many women and physicians remain unaware of the characteristic symptoms. Greater public and medical awareness is critical for identifying cases early.

All individuals should perform regular self-breast exams and be evaluated promptly for any nipple changes. Healthcare providers must consider Paget’s disease as a possible cause of nipple rash, discharge, or irritation.

Early detection through screening and vigilance gives patients the greatest chance of successful treatment. Raising awareness about this rare manifestation of breast cancer makes a real difference in outcomes.

If you are concerned about potential nipple changes, do not delay in seeking medical advice. And advocate for yourself until you get the answers you need. The more informed patients and doctors are about Paget’s disease, the better the prognosis can be.

FAQs

What does Paget’s disease of the breast look like?

Signs include red, irritated, scaly, or flaky skin on or around the nipple. There may also be oozing, discharge, or bleeding. The nipple may flatten or become indented.

What causes the skin changes of Paget’s disease?

The underlying breast cancer cells originate in the milk ducts and spread into the nipple tissue, causing the characteristic rash, erosion, and other skin changes.

How is Paget's disease diagnosed?

Doctors make the diagnosis based on examination of the nipple skin changes, breast imaging tests to look for tumors, and biopsy of the affected skin.

Is Paget’s disease of the breast treatable?

Yes, treatment usually involves surgery along with some combination of radiation, chemotherapy, or hormone therapy. The prognosis is often good with early diagnosis.

What is the survival rate for Paget’s disease?

The 5-year survival rate approaches 100% for early stage disease confined to the nipple. More advanced disease has lower survival rates, but prognosis is improving all the time.

Disclaimer: This article is for informational purposes only and does not constitute medical advice. Always consult with a healthcare professional before starting any new treatment regimen.