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Introduction: Paget's disease of the breast is a rare condition which is usually associated with underlying ductal in situ or invasive carcinoma. The incidence of tumor recurrence after mastectomy with preservation of the nipple-areola complex (NAC) is still unclear. Case Report: A 66-year-old woman diagnosed with a high-grade ductal carcinoma in situ underwent a nipple-areola sparing mastectomy, breast reconstruction with submuscular prosthesis, and adjuvant tamoxifen treatment. Eight years following breast surgery, the patient presented with an eczematoid lesion in the NAC. Histopathological exam was consistent with Paget's disease of the breast. Considerations: Only 14 cases of Paget's disease after nipple-areola sparing mastectomy have been published in the literature. The mean patient age reported was 48.8 years and disease occurred after a mean period of 44.6 months.

Objetivos: Avaliar a sobrevida de uma série de pacientes com doença de Paget na mama. Métodos: Estudo observacional, retrospectivo, descritivo. Foram coletados os dados através de prontuário eletrônico e obtidas as seguintes variáveis: idade, histologia do tumor, tamanho do tumor, grau de diferenciação, invasão linfática, invasão vascular, invasão neural, presença ou não de comprometimento de linfonodos axilares, perfil imunoistoquímico, tratamentos realizados, recidiva e seguimento. Resultados: Dos 301 casos de câncer de mama atendidos, foram identificadas 6 pacientes com doença de Paget na mama. A sobrevida global, com um seguimento médio de 54 meses, foi de 100%. Todas estão sem doença em atividade. O perfil histoquímico mais frequente foi negativo para receptores de estrógeno e de progesterona, e positivo para o HER-2/neu. Não foi identificado comprometimento metastático axilar e/ou a distância. Conclusões: A sobrevida global foi de 100%, com um seguimento médio de 54 meses. Essa alta taxa deve-se à ausência de comprometimento metastático axilar e/ou a distância em nossa série.PALAVRAS-CHAVE: Doença de Paget mamária; mama; doenças mamárias; neoplasias da mama; eczema. RESUMO ABSTRACTObjective: To evaluate the survival of a series of patients with Paget's disease of the breast. Methods: Observational, retrospective and descriptive study. Data were collected through electronic medical records; the following variables were obtained: age, tumor histology, tumor size, degree of differentiation, lymphatic invasion, vascular invasion, neural invasion, presence or not of potential involvement of axillary lymph nodes, immunohistochemical profile, treatments performed, recurrence, and follow-up. Results: Of the 301 cases of assisted breast cancer, six patients were identified with Paget's disease of the breast. The overall survival, with a mean follow-up of 54 months, was 100%. All individuals are free of disease activity. The most common histochemical profile was negative for estrogen and progesterone receptors, and positive for HER-2/neu. Axillary and/or distal metastatic involvement was not identified. Conclusions: Overall survival was 100%, with a mean follow-up of 54 months. This high rate is due to the absence of axillary and/or distal metastatic involvement in our series.