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.

Adenoid cystic carcinoma of head and neck. A 5-year retrospective study: Experience in a single third-level reference center.

INTRODUCTION: Adenoid cystic carcinoma (ACC) is a rare tumor, with an annual incidence of 3-4.5 cases/million. MATERIALS AND METHODS: We conducted a descriptive and retrospective study. We included patients diagnosed with ACC of head and neck of a single third-level referral center, from January 2008 to December 2013. RESULTS: A total of 23 patients were included in the study, 10 males (43.5%) and 13 females (56.5%) (F:M ratio 1.3:1). Median age was 52 years. Localization was predominantly observed in the oral cavity (26%) followed by the palate (17.4%). At presentation, 15 patients (65.2%) had clinical Stage II. The most frequent histology was the cribriform type in 10 patients (43.5%). Following resection, positive margins were observed in 15 (65.2%), neural invasion in 8 (34.8%), and lymphovascular invasion in 2 (8.7%) patients. The median time to recurrence in our population was 23 months. Recurrence at 5 years was observed in 15 patients (65%). At 2 years, recurrence was predominantly local in 7 patients (46%); whereas after 2 years, it was predominantly pulmonary 6 patients (40%). Overall, 5 year-survival was 78%. CONCLUSIONS: Cystic adenoid carcinoma is characterized by a high recurrence rate. Nevertheless, it has a high overall 5-year survival, which justifies an aggressive treatment.

ANTECEDENTES: El carcinoma adenoide quístico es un tumor raro, con una incidencia anual de 3-4.5 casos por millón. MATERIALES Y MÉTODOS: Estudio descriptivo, retrospectivo, que incluyó pacientes con carcinoma adenoide quístico de cabeza y cuello de un centro de referencia de tercer nivel, desde enero de 2008 hasta diciembre de 2013. RESULTADOS: Se incluyeron 23 pacientes, 10 hombres (43.5%) y 13 mujeres (56.5%) (relación 1.3:1), con una media de edad de 52 años. Predominó en la cavidad bucal (26%), seguida del paladar (17.4%). Al diagnóstico, 15 pacientes (65.2%) tenían estadio clínico II. La histología cribiforme fue la más frecuente (10 pacientes; 43.5%). Posterior a la cirugía, se observaron márgenes positivos en 15 (65.2%), invasión neural en 8 (34.8%) e invasión linfovascular en 2 (8.7%) pacientes. La media de recurrencia fue de 23 meses. La recurrencia a 5 años se observó en 15 pacientes (65%). A los 2 años predominó la recurrencia local (7 pacientes; 46%); después de los 2 años predominaron las metástasis pulmonares (6 pacientes; 40%). La supervivencia a 5 años fue del 78%. CONCLUSIONES: El carcinoma adenoide quístico se caracteriza por una alta tasa de recurrencia. Sin embargo, tiene una alta supervivencia global a 5 años, lo que justifica un tratamiento agresivo.