Extramammary Paget disease (EMPD) is a rare dermatologic condition that frequently presents in areas where apocrine sweat glands are abundant, most commonly the vulva, although perineal, scrotal, perianal, and penile skin may also be affected. Lesions clinically present as erythematous, well-demarcated plaques that may become erosive, ulcerated, scaly, or eczematous. Extramammary Paget disease has a female predominance and usually occurs in the sixth to eighth decades of life. Professionals disagree about many aspects of EMPD, for example, the prevalence of concurrent vulvar adenocarcinoma or invasive EMPD, association with regional and distant cancers, and recurrence rates following surgical excision. Early recognition is imperative because the diagnosis is frequently delayed and there is a high incidence of associated invasive disease.
Park et al. report their experience in the management of extramammary Paget’s Disease (EMPD) of the penoscrotal region and specifically compare outcomes among cohorts of men with the disease who either did or did not undergo mapping biopsies prior to their definitive surgical procedure. The rationale for the study and this comparison is that Paget’s disease initially spreads insidiously through the epidermis, sometimes in a single-cell fashion, and establishing the diagnosis can be very difficult subsequent to intraoperative frozen sections. Thus, several studies have described the use of outpatient mapping biopsies under more permanent section pathology techniques to facilitate the diagnosis and to ‘clear’ the surgical margins (references 19–21 in the article). This should theoretically lead to a lower incidence of positive frozen section margins intraoperatively, a lower incidence of positive permanent section margins, and lower recurrence rates for patients.
Mohs surgery with cytokeratin-7 immunohistochemistry staining effected complete removal of extramammary Paget disease and resulted in a 5-year, 95% recurrence-free cure rate.
The results are significantly better than the often-cited 77% cure rate seen with Mohs surgery alone, Dr. Ali Alexander Damavandy said at the annual meeting of the American College of Mohs Surgery.
“These are statistically significant and clinically substantial results,” said Dr. Damavandy, a procedural dermatology fellow at the University of Pennsylvania, Philadelphia. “With this method you can tell a patient that in 5 years, he has a 95% chance of still not having the tumor. The high recurrence-free rate we have seen supports the view that Mohs surgery with cytokeratin-7 [CK-7] immunohistochemistry should be considered the curative treatment of choice for both primary and recurrent extramammary Paget disease of the skin.”