Vulvar Paget Disease

OBJECTIVE: Our aim was to add information to the current literature on vulvar Paget disease by reviewing a consistent number of patients who have been all diagnosed, treated, and followed up by the same group of physicians at a single medical institution. METHODS: Clinical, surgical, histological, and follow-up data of 34 patients (mean [SD] age at diagnosis = 68.7 [10.1] years) with vulvar Paget disease were reviewed during a 27-year period. RESULTS: Primary symptoms were itching (76.5%) and burning (58.8%). Clinical manifestations were present for a mean (SD) of 17.8 (7.2) months before the diagnosis was made. Multifocal lesions were observed in 17 patients (50%) and were associated with a delay in diagnosis exceeding 12 months (p = .03). Of the patients, 10 (29.4%) presented a history of malignancy in other sites. Surgery with various extent of resection was performed as primary treatment in all patients. Definitive histological examination revealed positive surgical margins in 15 cases (44.1%), stromal invasion in 4 (11.7%), and associated adenocarcinoma in 2 (5.9%). Of the patients, 6 (17.6%) underwent reconstructive technique at their primary surgery or radicalization. During a mean (SD) follow-up of 76.9 (51.3) months, 15 patients (44.1%) experienced local recurrence (1 recurrence in 29.4%, 2 recurrences in 5.9%, and 3 recurrences in 8.8%). First recurrence appeared after a mean (SD) time of 45.7 (25.1) months and was associated with multifocal lesions (p = 0.005) and surgical margins involvement (p = 0.03). One patient (2.6%) died of the disease. CONCLUSIONS: Vulvar Paget disease is a chronic disease with high recurrence rate and low mortality. Early diagnosis, minimal surgery with free margins, and long-term follow-up are the cornerstones of treatment.