[ XXXX : five cases report].

@entity1 @entity712 @entity33835 @entity5 @entity4261 @entity189 @entity420
AIM: Extramammary Paget's disease is a rare @entity5 , often associated with a synchronous or metachronous underlying skin or @entity5 . The aim of this study was to evaluate retrospectively the results we got in 5 cases of @entity4261 and to revise what literature have reported about this issue. @entity1 AND METHODS: Five @entity1 with perianal EMPD were consecutively treated in our Division between March 1996 and December 2006. In 3 cases the disease was limited to the epidermidis, in one case with multiple recurrences there was dermal infiltration, and one @entity1 had a low @entity420 with @entity33835 phenomenon. The surgical treatment we performed in all @entity1 was a wide perianal excision, followed by reconstruction with cutaneous grafts; the resection of @entity420 was carried out using the transanal approach. In two cases we performed a temporary stoma with the sigmoid colon to help the wound healing. RESULTS: We didn't record any complication neither postoperative nor at long time. Two @entity1 developed a local recurrence, but none of our @entity1 showed distant metastases. Four @entity1 are alive and free from disease and one, who developed a multiple local recurrences, died for @entity712 . DISCUSSION: The studies available in literature clearly distinguish between a primary EMPD (intraepidermal/intradermal) and secondary disease which is associated with @entity420 and is thought to be a @entity33835 phenomenon, while few informations can derive from those cases in which the disease is associated with an underlying cutaneous @entity189 . Disease tend to relapse even after a radical surgery and can have metastatic spread, also in intraepidermal form. CONCLUSIONS: Perianal EMPD is a complex disease, difficult to recognize and the association with synchronous or @entity5 imposes long term clinical and instrumental follow up.
