Extramammary Paget disease (EMPD) is a very rare form of intraepithelial adenocarcinoma. We report the cases of EMPD diagnosed in a referral hospital over a 5-year period from 2009 to 2013. Ten cases of EMPD were diagnosed in 4 men and 6 women aged between 50 and 79 years. Erroneous clinical diagnoses led to diagnostic delays of 1 to 5 years in all cases. Six patients had in situ lesions and dermal invasion was observed in the other 4 cases. Four patients had a second neoplasm and 2 of the patients with invasive EMPD died. Because EMPD is rare and its clinical presentation is nonspecific, a high degree of clinical suspicion is required to avoid delays in diagnosis and treatment.
Keywords: Adenocarcinoma; Enfermedad de Paget extramamaria; Extramammary Paget disease; Rare tumor; Tumor raro.
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