Enfermedad de Paget extramamaria: 10 casos / Extramammary Paget Disease: A Report of 10 Cases

RESUMEN

La enfermedad de Paget extramamaria (EPEM) es una forma extremadamente rara de adenocarcinoma intraepitelial. Presentamos los casos de EPEM diagnosticados en los últimos 5 anos en un hospital de tercer nivel (2009-2013). Se trata de 10 casos, 4 varones y 6 mujeres, con edades comprendidas entre los 50-79 anos. Todos ellos sufrieron un retraso diagnóstico de uno a 5 anos debido a sospechas diagnósticas erróneas. Seis casos correspondieron a EPEM in situ y en 4 existía invasión dérmica. Se localizó 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 (AU)

ABSTRACT

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 (AU)