Metastatic extramammary Paget's disease of the scrotum

Background. - Extra-mammary Paget's disease (EMPD) is currently considered a slowly progressing in situ adenocarcinoma. It is much more rarely associated with an invasive underlying adenocarcinoma than in mammary sites, where this association is almost always seen. However, an invasive course is to be feared where EMPD has been progressing for several years. Patients and methods. - A 59-year-old man consulted for treatment of scrotal Paget's disease present for over 15 years and treated with topical emollients alone. Two infiltrated areas had recently appeared and their excision led to a diagnosis of invasive Paget's disease at these two points. Metastasis occurred 10 months later and rapidly proved fatal. Discussion. - Until now, non-invasive EMPD has been considered a fairly non-aggressive disease due to its indolent progression. However, it is characterised by considerable risk of relapse following surgical excision, regardless of the surgical margin. Despite a good prognosis at the intraepithelial stage, the rising life expectancy of the population means a greater likelihood of an invasive course or of the appearance of an underlying adenocarcinoma, and management of this condition can thus no longer be neglected. The present clinical case underscores the need for closer surveillance of the disease as of the intraepithelial stage. (C) 2012 Elsevier Masson SAS. All rights reserved.