Background & objective: Vulvar Paget's disease is a rare disease. It has a relatively high misdiagnosis rate and there are still controversies regarding its treatment. The objective of this study was to investigate its clinical features and summarize the experience of the management of this disease in order to gain a better acknowledge of this rare disease and improve its cure rate.
Methods: The clinical records of 8 cases of vulvar Paget's disease admitted in Cancer Center, Sun Yet-sen University from January 1964 to December 2001 were analyzed.
Results: The average age of 8 cases was 66.5 years, and the mean time from the onset of the disease to diagnosis was 5 years. Pathologically, intraepithelial Paget's disease was the commonest (5/8), followed by invasive Paget's disease (2/8) and Paget's disease with underlying adenocarcinoma (1/8). Eight cases underwent altogether 10 times of surgery, and radical vulvectomy was the most frequently used procedure (6/10). One case of Paget's disease with underlying adenocarcinoma died 21 months after surgery and 1 case of intraepithelial Paget's disease died of other etiology, 5 cases achieved long-term disease free survival, 1 case developed recurrence.
Conclusion: Surgery is the first choice for the patients of vulvar Paget's disease. For those patients whose lesions are extensive,or old aged,or not suitable for long-term follow-up, a radical vulvectomy is more preferable and a lower recurrence rate is expected.