Paget’s Disease of the Breast - Early Signs, Risk Factors, Diagnosis, and Treatment Explained

Paget’s disease of the breast is a rare type of breast cancer that affects the skin of the nipple and the darker area around it (areola). Unlike other breast cancers that form a lump deeper in the breast tissue, this cancer often starts in the milk ducts and spreads to the surface of the nipple and areola.

It usually appears as a skin change, such as redness, flaking, or itching of the nipple, often mistaken for eczema or other skin conditions. Because of this, many patients experience delays in diagnosis.

Paget’s disease of the breast accounts for about 1–4% of all breast cancer cases. Most patients also have underlying ductal carcinoma in situ (DCIS) or invasive breast cancer in the same breast. Early detection matters because if the disease is caught when it is still limited to the nipple and ducts, treatment is simpler and survival rates are higher.

What Are the Types of Paget’s Disease of the Breast?

Doctors often describe Paget’s disease based on whether another type of breast cancer is present:

• Paget’s disease with DCIS (ductal carcinoma in situ): The cancer cells are confined to the milk ducts and have not spread deeper. This is considered an early form of breast cancer.
• Paget’s disease with invasive breast cancer: In this case, cancer cells have spread beyond the milk ducts into nearby breast tissue. This is a more advanced form and may also spread to lymph nodes.

What Are the Causes of Paget’s Disease of the Breast?

The exact cause is not fully understood, but the most accepted explanation is the “epidermotropic theory” (migration of malignant ductal cells into the nipple epidermis). According to this, cancer cells from the milk ducts migrate up to the nipple skin, causing visible changes.

Like other breast cancers, the causes are linked to a combination of genetic, hormonal, and lifestyle factors, rather than a single trigger.

What Are the Risk Factors for Paget’s Disease of the Breast?

Since most cases occur along with other forms of breast cancer, the risk factors are similar to those of breast cancer in general. These include:

• Age: Most patients are over 50 years old.
• Gender: Women are most commonly affected, but rare cases can occur in men.
• Family history of breast cancer: Having close relatives with breast or ovarian cancer raises the risk.
• Genetic mutations: BRCA1, BRCA2, and other cancer-related genes may increase susceptibility.
• Hormone therapy: Long-term use of combined estrogen-progestin therapy after menopause may raise risk.
• Radiation exposure: Previous chest radiation increases risk.
• Lifestyle factors: Obesity, lack of exercise, high alcohol consumption, and smoking are associated with higher breast cancer risk.

What Are the Symptoms of Paget’s Disease of the Breast?

Paget’s disease often mimics skin conditions, which can make it tricky to identify.

Common symptoms include:

• Redness, flaking, or scaly skin on the nipple or areola
• Itching, tingling, or burning around the nipple
• Flattened or inverted nipple
• Discharge from the nipple (sometimes bloody or yellowish)
• A lump in the breast (in about half of patients)
• Thickened skin on the nipple or areola

If you notice these changes in one breast, especially if they don’t improve with creams or ointments, it’s important to see a doctor promptly.

How Is Paget’s Disease of the Breast Diagnosed?

Doctors use a combination of tests to detect and confirm Paget’s disease:

Physical examination: Checking the nipple, areola, and rest of the breast for changes.

Mammogram: X-ray imaging of the breast to detect underlying cancer. However, Paget’s disease is sometimes difficult to see on mammograms.

Breast ultrasound: Helps find hidden lumps or abnormalities.

Breast MRI: Provides detailed images and is especially useful if mammogram results are unclear.

Biopsy: A sample of nipple skin or underlying tissue is tested under a microscope. This is the definitive test to confirm diagnosis.

Staging of Paget’s Disease of the Breast

Staging tells doctors how far the cancer has spread, while grading describes how aggressive the cancer cells look under a microscope. Paget’s disease is staged according to the underlying cancer (DCIS or invasive). If only DCIS is present, it is considered Stage 0. If invasive disease exists, staging follows tumor size, nodal status, and metastasis.

Stage 0 (DCIS): Cancer cells are confined to ducts; Paget’s disease is only in nipple/areola skin.

Stage I–II: Small invasive cancer with limited spread to nearby lymph nodes.

Stage III: Larger tumor with spread to multiple lymph nodes or surrounding breast skin.

Stage IV: Cancer has spread to distant organs (lungs, liver, bones, etc.).

The stage of cancer directly impacts treatment choices and prognosis.

What Are the Treatment Options for Paget’s Disease of the Breast?

Treatment depends on whether other breast cancer is present and how advanced it is.

Surgery

• Mastectomy: Removal of the entire breast, including nipple and areola, often recommended if invasive cancer is present.
• Breast-conserving surgery (lumpectomy): Breast-conserving surgery may include central excision (removal of nipple–areola with underlying tissue) often combined with adjuvant radiation. Breast reconstruction techniques (such as nipple and areola tattooing) may be used for better cosmetic outcomes.
• Sentinel lymph node biopsy: Checks whether cancer has spread to the lymph nodes near the breast. Sentinel lymph node biopsy is standard for axillary staging. If one or two sentinel nodes are positive, many women can avoid full axillary dissection and instead receive radiation, depending on tumor characteristics.

Radiation Therapy

• May be recommended after breast-conserving surgery for Paget's disease of the breast to reduce the risk of cancer recurrence, especially if an underlying breast cancer is present. It uses high-energy X-rays to destroy any remaining cancer cells.

Medical Treatments

• Chemotherapy: Drugs given intravenously or orally to kill cancer cells, typically as adjuvant therapy (after surgery). Recommended if invasive cancer or lymph node involvement exists.
• Hormone therapy: If the cancer is hormone receptor-positive, medications like tamoxifen or aromatase inhibitors may be used. If HER2-positive, drugs like trastuzumab (Herceptin) may be included in treatment.

Proton Therapy: When Is It Applicable?

Available at Apollo Proton Cancer Centre, proton therapy uses protons instead of X-rays to deliver radiation more precisely to cancer cells. This allows doctors to target the tumor while minimizing damage to healthy surrounding tissue, including the heart and lungs.

Proton therapy is not routinely used for Paget’s disease itself, but may be considered in select breast cancer patients:

• When the tumor is located close to critical organs such as the heart or lungs, especially in left-sided breast cancers with internal mammary node involvement.
• In younger patients, to reduce long-term side effects from radiation exposure.
• For patients who have already received radiation to the chest and require additional treatment.
• In complex cases where precision is essential to reduce radiation-related complications.

While proton therapy is not required for all patients with Paget's disease of the breast, it can be especially beneficial in selected cases where traditional radiation may pose higher risks.

What is the Prognosis for Paget’s Disease of the Breast?

Survival rate depends on whether other breast cancer is present:

• Paget’s disease with DCIS only: Excellent prognosis. The 5-year survival rate is above 95%.
• Paget’s disease with invasive cancer: Prognosis depends on stage, receptor/HER2 status, and treatment response. Early-stage invasive cancers may have a 5-year survival of 80–90%, while advanced stages have lower survival rates.

Other factors affecting prognosis include age, tumor size, hormone receptor status, HER2 status, and overall health.

Screening for Paget’s Disease of the Breast

There is no specific screening for Paget’s disease. However, regular breast cancer screening helps detect it early:

• Mammograms: Women aged 40 and older should get regular mammograms, which can spot early signs of cancer, even before it can be felt.
• Breast Self-exams: Regular breast self-examinations are encouraged as they can help detect changes in the breast.
• Genetic testing: High-risk women, including those with a strong family history of cancer, may consider BRCA testing.
• Lifestyle changes: Maintain a healthy optimal weight, exercise regularly, avoid alcohol and smoking.

For International Patients

People from around the world come to Apollo Hospitals for the treatment of Paget’s disease of the breast. Our international patient services team will guide you all the way from seeking the first virtual connect all the way to treatment in India and then returning home post treatment.

Services include:

• Medical opinions and scheduling
  • Pre-arrival medical review of reports and imaging.
• Travel and logistics
  • Assistance with visa invitation letters, airport transfers, and nearby accommodation options.
  • Dedicated international patient coordinators to guide through each step.
• Language and cultural support
  • Interpreter services in multiple languages.
  • Clear, simple explanations at every stage with written care plans.
• Financial coordination
  • Transparent treatment estimates and packages when possible.
  • Support with international payment methods and insurance coordination.
• Continuity of care
  • Shared records, imaging, and treatment summaries for home doctors.
  • Telemedicine follow-ups for convenience after returning home.

Frequently Asked Questions (FAQs)

1. Is Paget’s disease of the breast curable?

Yes. If detected early and treated promptly, Paget’s disease, especially when limited to DCIS, can be cured.

2. What is the survival rate for Paget’s disease of the breast?

The survival rate is very high (above 95%) when no invasive cancer is present. With invasive cancer, survival depends on stage and response to treatment.

3. Can Paget’s disease of the breast come back?

Yes. Recurrence is possible, especially if invasive cancer was present. Regular follow-up and screening reduce the chances of late detection.

4. What are the side effects of treatment?

Side effects depend on treatment type. Surgery may cause scarring; radiation may lead to fatigue or skin irritation; chemotherapy can cause hair loss, nausea, or low immunity; hormone therapy may cause hot flashes or joint pain.

5. How long is the recovery time after surgery?

Recovery from breast-conserving surgery usually takes 2–3 weeks, while mastectomy may take 4–6 weeks. If reconstruction is done, healing may take longer.

6. How do I know if my nipple changes are cancer?

If symptoms such as redness, scaling, or discharge do not improve with regular creams or medicines, it’s important to see a breast specialist. A biopsy is the only way to confirm.