Clinical analysis of 13 cases of vulvar Paget's disease.

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 13 cases of vulvar Paget's disease admitted in Cancer Center of Sun Yat-sen University and the Third Affiliated Hospital of Sun Yat-sen University from January 1964 to December 2006 was analyzed. Results The average age of 13 cases was 61 years and the mean time from the onset of the disease to diagnose was 2 years. Pathologically intraepithelial Paget's disease was the commonest (10/13) followed by invasive Paget's disease (2/13) and Paget's disease with underlying adenocarcinoma (1/13). Three patients underwent simple vulvectomy. Three patients underwent wide local excision. Four patients underwent radical vulvectomy and three patients underwent radical vulvectomy with unilateral lymph node dissection and eight patients remain free of disease and three had recurrence of Paget's disease, one died of disease and finally one died of other etiology. Conclusion Surgery is the first choice for the patients of vulvar Paget's disease. Patients who are not suitable for long term follow up, extensive lesions or old aged are more preferable for radicaI vulvectomy and also the expectation rate of lower recurrence. [ABSTRACT FROM AUTHOR]