Neue Optionen bei der Therapie des Morbus Paget der Vulva

Objectives: Paget's disease of the vulva as an extramammary form is a rare and hard to treat epidermal neoplasia. In 12% underlying invasive Paget's carcinoma and in 4% associated adenocarcinoma wil be found. After surgical intervention a relapse is seen in 30%. The aim of this study was to find less invasive or mutilating therapy strategies than re-excision, x-ray or chemotherapy. Successful treatment of Paget's disease of the vulva with Imiquimod was described in a few case reports. Methods: Data of fourteen patients from the Medical School Hanover and the Department of Gynecology and Obstetrics of the urban clinic Wolfsburg with Paget's disease or carcinoma of the vulva were reviewed between September 1992 and March 2006. The primary therapy was surgery of the clinical lesions. Recurrent Paget's disease was treated by excision again and/or by a local therapy with Imiquimod 5% ointment. Results: In ten cases primary Paget's disease was diagnosed. One patient with an earlier carcinoma of the bladder showed secondary Paget's disease with a minimally invasive Paget's carcinoma. Three women showed an invasive Paget's carcinoma of the vulva, one of them with an associated adenocarcinoma. Treatment of recurrent Paget's disease (n= 5) was surgery or therapy with the immune stimulator Imiquimod (n = 3). Both total and partial remissions were noted. Conclusion: Surgery remains the primary therapy for Paget's disease of the vulva. For the treatment of recurrent Paget's disease new options are available based on the use of Imiquimod or Trastuzumab.