Perianal Paget disease treated definitively with radiotherapy

Abstract

Extramammary Paget disease (empd) is a relatively rare cutaneous disorder described as an apocrine gland tumour occurring in both a benign and a malignant form with metastatic potential. The areas of the body affected are the vulva, perianal region, penis, scrotum, perineum, and axilla, all of which contain apocrine glands. When empd affects the perianal region, it is called perianal Paget disease (ppd). All forms of empd, including ppd, are typically treated by wide surgical excision. Perianal Paget disease usually occurs later in life in patients who are often poor surgical candidates, but the available literature is scarce regarding other treatment modalities, including definitive radiotherapy. We contend that ppd can be safely and effectively treated with radiotherapy, and here, we present the case of a 75-year-old woman with ppd who was successfully so treated. A brief review of the literature concerning the diagnosis, natural history, and treatment of ppd is also included.

Keywords: Perianal Paget disease; extramammary Paget disease; radiotherapy.

FIGURE 1

Extensive perianal Paget’s disease in the perineum.

FIGURE 2

Early response observed by the 4th week on radiotherapy.

FIGURE 3

(A,B) Complete regression at 5 years post radiotherapy, with notable telangiectasia and fibrosis.