Extramammary Paget Disease: A Report of 10 Cases

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.

ResumenLa enfermedad de Paget extramamaria (EPEM) es una forma extremadamente rara de adenocarcinoma intraepitelial. Presentamos los casos de EPEM diagnosticados en los últimos 5 años en un hospital de referencia (2009-2013). Se trata de 10 casos, 4 varones y 6 mujeres, con edades comprendidas entre los 50-79 años. Todos ellos sufrieron un retraso diagnóstico de uno a 5 años debido a sospechas diagnósticas erróneas. Seis casos correspondieron a EPEM in situ y en 4 existía invasión dérmica. Existió una segunda neoplasia en 4 pacientes y 2 con EPEM invasiva fallecieron. La baja incidencia de esta enfermedad y su clínica inespecífica nos debe alertar sobre la necesidad de establecer una alta sospecha clínica para evitar retrasos diagnósticos y terapéuticos.