Clinicopathologic Study of Vulvar Paget’s Disease in China

OBJECTIVE: To assess the clinicopathologic characteristics of patients with vulvar Paget's disease who were admitted in our hospital or of cases reported in a Chinese journal. METHODS: The age, disease course, clinical manifestation, pathologic diagnosis, treatment, and follow-up data of patients with vulvar Paget's disease were reviewed. RESULTS: There were 85 cases of vulvar Paget's disease reviewed. The mean age of patients with vulvar Paget's disease was 64.4 years, and their mean disease course was 52.36 months. The patients' primary clinical manifestations were pruritus. Treatment included simple vulvectomy, wide local excision, partial vulvectomy, radical vulvectomy with/without groin dissection, and radiotherapy. Intraepithelial Paget's disease was the most common pathology type (61.5%) followed by invasive Paget's disease (20.0%). There were 7 patients who had a history of secondary malignancy. The mean follow-up period was 43.6 months. The recurrence rate was as high as 43.5%. No significant relationships between margin status, lymph node involvement, pathology type, and recurrence were found (p > .05). CONCLUSIONS: Vulvar Paget's disease has a high local recurrence risk and a long-term follow-up is required.