Radiotherapy for extramammary Paget disease of the anogenital region

Extramammary Paget disease is a rare condition that most commonly affects the anogenital region in the elderly. The treatment of choice has been surgical excision of the affected area with adequate depth and lateral margins, criteria that cannot always be fulfilled, especially when the vulva, anal canal, or penis are involved. More recently radiotherapy has been suggested as a suitable treatment when surgical excision or other modalities are not appropriate. We report a case of anogenital extramammary Paget disease and the clinical response to treatment with radiotherapy. The aim of this article is to review relevant aspects of radiotherapy as a first-choice curative treatment in specific situations of anogenital extramammary Paget disease in situ.