What is the process for getting a nipple biopsy to diagnose possible Paget's disease?

Nipple Biopsy for Suspected Paget's Disease of the Breast

For suspected Paget's disease of the breast, a full-thickness surgical biopsy of the nipple-areolar complex that includes the epidermis and at least a portion of any clinically involved area is essential for diagnosis, as this condition can be occult on imaging studies. 1, 2

Initial Evaluation Process

1. Clinical Assessment

   • Look for characteristic symptoms:
     • Eczema-like changes of nipple/areola
     • Bleeding or ulceration
     • Itching of the nipple
     • Nipple excoriation and scaling
     • Nipple discharge 1, 2

2. Imaging Studies

   • For women under 30 years:

     • Ultrasound first
     • Consider diagnostic mammogram 1, 2

   • For women 30 years and older:

     • Bilateral diagnostic mammogram AND ultrasound 1, 2

   • Important note: A negative mammogram does NOT exclude Paget's disease, as it can be occult on imaging in up to 50% of cases 2, 3

3. Biopsy Procedure

   • Type of biopsy: Full-thickness surgical biopsy of the nipple-areolar complex 1, 2
   • What it should include:
     • Complete epidermis
     • At least a portion of clinically involved nipple-areolar complex
     • Adequate depth to evaluate for underlying disease 1, 2
   • Alternative: Punch biopsy of skin/nipple can be performed if imaging findings are BI-RADS category 1-3 1

Post-Biopsy Management

1. If biopsy confirms Paget's disease:

   • Breast MRI is recommended to:
     • Define extent of disease
     • Identify additional disease elsewhere in the breast 1, 2, 4
   • This is critical as 80-90% of Paget's disease cases are associated with underlying breast cancer (DCIS or invasive) 1, 2

2. If biopsy is negative but clinical suspicion remains high:

   • Reassess clinical and pathologic correlation
   • Consider:
     • Breast MRI
     • Repeat biopsy
     • Consultation with breast specialist 1

Important Considerations

• Do not delay diagnosis with antibiotics or topical treatments for presumed dermatologic conditions 1
• Exfoliative cytology may be useful but a negative finding does not exclude Paget's disease 5
• Paget's disease is frequently associated with underlying DCIS in the lactiferous ducts or invasive cancer elsewhere in the breast 1, 2, 3
• The associated cancer may be located at least 2 cm from the nipple-areolar complex 3

Treatment Following Diagnosis

Based on biopsy and imaging results, treatment options include:

• Breast-conserving surgery: Removal of entire nipple-areolar complex with negative margins, plus removal of any peripheral cancer, followed by radiation therapy 1, 2
• Mastectomy: Remains appropriate, especially with extensive disease 1, 2
• Axillary staging: Not necessary for Paget's with DCIS only; required for invasive disease 1, 2

Common Pitfalls to Avoid

• Dismissing nipple changes as benign dermatologic conditions without thorough evaluation 2, 5
• Relying solely on imaging without biopsy 1, 2, 3
• Delaying diagnosis with empiric treatments 1
• Failing to perform MRI when initial imaging is negative 1, 2, 4

Remember that early diagnosis and appropriate treatment significantly improve outcomes for patients with Paget's disease of the breast 2, 6.