What are the NCCN (National Comprehensive Cancer Network) guidelines for observation of Paget's disease of the vulva?

NCCN Guidelines for Observation of Paget's Disease of the Vulva

For patients with Paget's disease of the vulva, surveillance should follow the standard NCCN guidelines for vulvar cancer with history and physical examination every 3-6 months for 2 years, every 6-12 months for 3-5 years, and then annually thereafter. 1

Surveillance Protocol

Recommended Follow-up Schedule

• First 2 years: History and physical examination every 3-6 months
  • Patients with high-risk disease: Consider more frequent assessment (every 3 months)
  • Patients with low-risk disease: Consider less frequent assessment (every 6 months)
• Years 3-5: History and physical examination every 6-12 months
• Beyond 5 years: Annual history and physical examination

Key Components of Follow-up Visits

• Thorough vulvar examination to detect local recurrence
• Cervical/vaginal cytology screening as indicated for detection of lower genital tract dysplasia
  • Note: Value in detecting recurrent cancers is limited
  • May include HPV testing when appropriate
• Imaging only as indicated by suspicious findings or symptoms
  • Options include chest/abdomen/pelvis CT, PET/CT, or pelvic MRI
• Laboratory assessment (CBC, BUN, creatinine) only as indicated by symptoms or examination findings

Special Considerations for Paget's Disease

Paget's disease of the vulva has several unique characteristics that affect surveillance:

1. High recurrence rates: Recurrence is common regardless of margin status during initial surgery 2

   • Studies show approximately 60% recurrence rate even with negative surgical margins 2
   • Long-term surveillance is essential as recurrences can occur beyond 5 years 1

2. Association with underlying malignancies:

   • Up to 20-30% of cases may be associated with an underlying carcinoma 3
   • Approximately 58% of patients with recurrent disease may have coexisting malignancy 2
   • Vigilance for symptoms of other malignancies is warranted

3. Margin status:

   • Negative margins do not guarantee prevention of recurrence 2
   • Patients should remain under surveillance for life regardless of initial margin status

Patient Education During Follow-up

During surveillance visits, provide education on:

• Symptoms of potential recurrence and vulvar dystrophy
• Importance of periodic self-examinations
• Lifestyle factors: obesity management, exercise, nutrition
• Sexual health (including vaginal dilator use and lubricants/moisturizers if needed)
• Smoking cessation
• Potential long-term effects of treatment 1

Management of Suspected Recurrence

If recurrence is suspected based on symptoms or examination:

1. Obtain appropriate imaging (CT scan of chest, abdomen, and pelvis)
2. Perform biopsy to confirm recurrence
3. Evaluate extent of disease to guide further management 1

The long-term follow-up is critical as studies have shown that recurrences can occur many years after initial treatment, with some series reporting recurrences beyond 5 years after initial therapy 1.