Management of perianal extramammary Paget’s disease involving the dentate line without abdominoperineal resection

Excerpt

Extramammary Paget’s disease (EMPD), first reported in 1889, commonly involves the vulva and perianal area [1‐3]. By definition, perianal EMPD describes the lesions that are located within 6 cm from the anus and below the dentate line [1]. The disease is very rare and even in a major tertiary referral center like Memorial Sloan Kettering Cancer Center where only 65 cases were reported in 60 years [4]. The true incidence of perianal EMPD is not known, but some estimates suggest it is approximately 20 % of all cases of EMPD [4, 5]. Surgical resection provides the best chance of curative treatment, but the multifocality of the disease is responsible for a high local recurrence rate [3]. Sometimes abdominoperineal resection with a permanent stoma may be considered in cases of extensive perianal involvement or involvement of the dentate line [4, 5]. Three females and three males with perianal EMPD were referred to our department since 2010, with a mean age at diagnosis of 66.7 years (range 55–75 years). Three patients were diagnosed with perianal EMPD only, and another three with recurrent perineal EMPD involving the perianal region. Mean time from the last treatment to recurrence was 3.0 years (range 1–5 years). The main presentation was perianal discomfort or pruritus, which progressively worsened over time. All three patients with recurrent perineal EMPD were female (all had had primary vulval EMPD, and one of them had also had concomitant vulval carcinoma). …