[Perianal Paget's disease: five cases report]

Extramammary Paget's disease is a rare neoplastic condition, often associated with a synchronous or metachronous underlying skin or visceral malignancies. The aim of this study was to evaluate retrospectively the results we got in 5 cases of perianal Paget's disease and to revise what literature have reported about this issue.

Five patients 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 patient had a low rectal adenocarcinoma with pagetoid phenomenon. The surgical treatment we performed in all patients was a wide perianal excision, followed by reconstruction with cutaneous grafts; the resection of rectal adenocarcinoma was carried out using the transanal approach. In two cases we performed a temporary stoma with the sigmoid colon to help the wound healing.

We didn't record any complication neither postoperative nor at long time. Two patients developed a local recurrence, but none of our patients showed distant metastases. Four patients are alive and free from disease and one, who developed a multiple local recurrences, died for heart failure.

The studies available in literature clearly distinguish between a primary EMPD (intraepidermal/intradermal) and secondary disease which is associated with anorectal adenocarcinomas and is thought to be a pagetoid phenomenon, while few informations can derive from those cases in which the disease is associated with an underlying cutaneous adnexal carcinoma. Disease tend to relapse even after a radical surgery and can have metastatic spread, also in intraepidermal form.

Perianal EMPD is a complex disease, difficult to recognize and the association with synchronous or metachronous malignancies imposes long term clinical and instrumental follow up.