Paget Disease of the Nipple: What It Is and How It Is Treated

Written by Medicover Team and Medically Reviewed by Dr Kovvuru Bhaskar Reddy , Oncologists

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1. Causes 2. Find a Doctor 3. Diagnosis 4. Treatment 5. FAQs

Paget's Disease of the Nipple is a rare form of breast cancer that affects the nipple and areola. It accounts for a small percentage of breast cancer cases and may occur alongside ductal carcinoma in situ (DCIS) or invasive breast cancer.

Despite its rarity, a diagnosis of Paget's Disease of the Nipple requires careful evaluation to determine whether an underlying breast cancer is present. Understanding its symptoms, diagnosis, treatment, and prognosis can support timely medical care.

What Are the Types of Paget's Disease of the Nipple?

Paget's Disease of the Nipple is generally classified based on whether the abnormal Paget cells are associated with an underlying breast cancer and whether invasive disease is present.

Determining the extent and type of associated breast cancer is important because it influences treatment decisions and helps doctors assess the overall outlook.

Paget's Disease With Ductal Carcinoma In Situ (DCIS): Paget's disease may occur alongside non-invasive cancer cells within the breast ducts.
Paget's Disease With Invasive Breast Cancer: Paget's disease may occur with invasive breast cancer that has spread beyond the breast ducts into surrounding breast tissue.
Paget's Disease Without Detectable Underlying Cancer: In some cases, Paget cells may be limited to the nipple and areola without an immediately identifiable underlying breast tumor.

What Are the Symptoms of Paget's Disease of the Nipple?

Identifying the symptoms of Paget's Disease of the Nipple is essential for early evaluation. The symptoms can be subtle and may initially resemble common skin conditions such as eczema or dermatitis.

Persistent or progressive nipple changes, particularly when they affect only one breast, should be assessed by a doctor.

Persistent redness, flakiness, or crusting of the nipple skin
Itching, burning, or tingling sensations around the nipple
Yellowish or bloody discharge from the nipple
Inversion or flattening of the nipple
Thickening or scaling of the nipple or areola
Presence of a palpable lump in the breast
Pain or tenderness in the nipple or breast

What Are the Causes of Paget's Disease of the Nipple?

The exact cause of Paget's Disease of the Nipple is not fully understood. It is generally associated with abnormal cancerous cells developing in the nipple and is frequently linked to an underlying breast cancer.

One commonly proposed explanation is that cancer cells from the milk ducts migrate toward the nipple and grow within the nipple skin. Genetic changes that promote abnormal cell growth are also believed to play a role.

Underlying breast cancer, including DCIS or invasive breast cancer
Migration of abnormal cells from the breast ducts to the nipple
Genetic mutations that contribute to abnormal breast cell growth
Other biological and environmental factors that may contribute to breast cancer development

When to See a Doctor?

Consult a doctor if nipple or breast changes persist, particularly if redness, scaling, crusting, discharge, or a lump does not improve with routine treatment. Early evaluation is important because Paget's Disease of the Nipple may be associated with an underlying breast cancer.

A breast surgeon or oncologist can evaluate suspicious nipple and breast changes. A dermatologist may also assess nipple skin changes, while a medical oncologist or radiation oncologist may be involved if breast cancer is confirmed.

Persistent redness, itching, scaling, or crusting of the nipple
Unusual nipple discharge, including bloody fluid
Changes in nipple shape or inversion
A new breast or nipple lump
Breast or nipple changes that do not improve with usual skin treatment

Get medical help promptly if:

Rapid worsening of nipple or breast changes
Ulceration or bleeding from the nipple
A new lump associated with nipple or skin changes
Significant or persistent breast pain with other concerning changes

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How Is Paget's Disease of the Nipple Diagnosed?

Accurate diagnosis of Paget's Disease of the Nipple involves a clinical examination, medical history, and diagnostic breast imaging. Because its symptoms can resemble benign skin conditions, further investigation is important when nipple changes persist.

A biopsy of the affected nipple or areola provides definitive confirmation by allowing the tissue to be examined for Paget cells. Additional tests may determine whether an underlying breast cancer is present.

Clinical Examination: The breast, nipple, and areola are examined for changes in skin appearance, discharge, lumps, or other abnormalities.
Mammography: Mammography can help detect underlying masses, calcifications, or other breast abnormalities.
Ultrasound: Breast ultrasound can evaluate areas of concern and help identify masses or other abnormalities.
Biopsy: A biopsy of the affected tissue provides a definitive diagnosis by identifying Paget cells under microscopic examination.
Additional Imaging: MRI or other imaging may be recommended when further assessment of the breast is clinically necessary.

Complications of Delayed Diagnosis

Delayed diagnosis of Paget's Disease of the Nipple can allow an associated breast cancer to progress. Identifying the condition early helps determine the extent of disease and allows appropriate treatment to begin sooner.

What Are the Treatment Options for Paget's Disease of the Nipple?

Treatment for Paget's Disease of the Nipple depends on whether an underlying breast cancer is present, the extent of the disease, and whether the cancer is invasive. A multidisciplinary breast cancer team develops the treatment plan based on these factors.

Treatment may involve surgery followed by additional therapies such as radiation, chemotherapy, or hormone therapy when indicated by the characteristics and stage of the underlying cancer.

Surgical Intervention

Mastectomy: Removal of the entire breast may be recommended when extensive or invasive breast cancer is present or when breast-conserving treatment is not appropriate.
Breast-Conserving Surgery: Also known as lumpectomy, this involves removing the affected tissue and an appropriate margin of healthy tissue while preserving as much of the breast as possible in selected patients.

Adjuvant Therapies

Radiation Therapy: Radiation therapy may be recommended after breast-conserving surgery and in selected other situations to reduce the risk of cancer recurrence.
Chemotherapy: Chemotherapy may be recommended when invasive breast cancer has features that indicate a benefit from systemic treatment.
Hormone Therapy: Hormone therapy may be used when the underlying breast cancer is hormone receptor-positive.
Targeted Therapy: Targeted medicines may be considered when the cancer has specific molecular characteristics, such as HER2 positivity.

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What Is the Prognosis and Long-Term Outlook for Paget's Disease of the Nipple?

The prognosis for Paget's Disease of the Nipple depends largely on whether an underlying breast cancer is present and whether the cancer is invasive. Early diagnosis and treatment generally provide a better outlook, particularly when the disease is limited and non-invasive.

Regular follow-up examinations and recommended breast imaging are important after treatment to monitor for recurrence and manage long-term breast health.

Emotional and Psychological Considerations

A diagnosis of Paget's Disease of the Nipple can cause emotional and psychological distress, particularly when it is associated with breast cancer. Support from healthcare professionals, counseling services, family, and patient support groups can help individuals cope with diagnosis and treatment.