Perianal Paget’s disease: case report and review of the literature

Abstract

A peri-anal skin lesion, often eczema-like and with symptoms of pruritus, that does not resolve after classical local therapy should be biopsied. We present a case of peri-anal extramammary Paget's disease (EMDP) and associated anal adenocarcinoma. Reviewing the literature, more than 30% of patients with EMDP present a second primary tumour in their past, present or future history. In Europe, the risk of developing a new primary tumour in patients with this condition is increased compared with the standard population. In cases of peri-anal Paget's disease (PPD), specific histochemical markers allow us to differentiate between a primary and a secondary form, the secondary one is strongly associated with colorectal and anal tumours. We provide information about the most commonly suggested therapy for PPD with or without associated malignancy and about the recommended follow-up.

Wide local excision of perianal Paget’s disease with gluteal flap reconstruction: an interdisciplinary approach

“…Diagnosis of PPD itself tends to be considered after a lack of response to local treatments (4,5). Since symptoms of PPD are similar to those seen in common anorectal problems such as hemorrhoids, anal fissures, and pruritus ani, PPD is commonly mistaken as one or more of those conditions (5).…”

“…PPD is a type of EMPD that specifically affects the tissues surrounding the anal region (3). Rates of synchronous or metachronous malignancies in PPD range from 33% to 86% (4). In EMPD, the location of other malignancies tends to be related to the location of the EMPD itself (4).…”

“…Primary PPD is described as a relatively benign, local disease (4). However, discontinuous spread and insufficient resection margins often lead to frequent local recurrence (4). Secondary PPD, also known as Pagetoid spread or Pagetoid phenomenon, is strongly Case Report on Colorectal Surgery Wide local excision of perianal Paget's disease with gluteal flap reconstruction: an interdisciplinary approach associated with colorectal and anal tumors, often from a downward spread of rectal adenocarcinoma (4,5).…”

“…However, discontinuous spread and insufficient resection margins often lead to frequent local recurrence (4). Secondary PPD, also known as Pagetoid spread or Pagetoid phenomenon, is strongly Case Report on Colorectal Surgery Wide local excision of perianal Paget's disease with gluteal flap reconstruction: an interdisciplinary approach associated with colorectal and anal tumors, often from a downward spread of rectal adenocarcinoma (4,5). Although differentiation between primary and secondary PPD is not always clear-cut, such differentiation can be achieved through immunohistochemical analysis (3)(4)(5) (Figure 1).…”

“…The origin of Paget's cells may be apocrine gland cells based on the observation that there is increased apocrine gland density within the lesions (5). There are two types of PPD: primary and secondary (1,4,5). Primary PPD is described as a relatively benign, local disease (4).…”