Clinical analysis of extramammary Paget’s disease of vulva

Objective: To review our experience in diagnosis, pathology, treatment, and prognosis of extramammary Paget’s disease of the vulva. Methods: Seven patients with vulvar Paget’s disease who were treated at the Cancer Hospital cAMS were retrospectively reviewed for the period from 1960 to 2002. The symptoms, location of disease, pathology, mode of treatment and current status of the patients were evaluated. Results: A total of seven women with Paget’s disease of the vulva were admitted to the Department of Gynecologic of Oncology. The mean age was 67.3 years (range 54–81 years). Vulvar lesion and pruritus were the common symptoms and it took average 3.4 years to confirm the diagnosis. All patients underwent surgery as initial treatment, vulvectomy was performed for 2 patients and radical vulvectomy plus groin node dissection for 5 patients. Two patients had invasive disease and four were associated with underlying adenocarcinoma. Five patients experienced recurrence, on average, 16.2 months after the surgery. Four of them underwent radiotherapy or chemotherapy and the other received no further treatment. The mean follow-up time was 41.1 months. Three patients, with invasive lesion or underlying adenocarcinoma were dead of the disease. Conclusion: There is a delay in the diagnosis of vulvar Paget’s disease. The major treatment is surgery. Recurrence is relatively common. Patients with invasive disease or underlying carcinoma have a poor prognosis.