Pigmented mammary paget’s disease mimicking melanoma in male patients: a case report and review of the literature

Abstract

Background: Pigmented mammary Paget's disease (PMPD) of the male breast is extremely rare, and only few cases have been reported to date. Due to the scarcity of the disease, diagnosis as well as treatment is usually delayed. Men tend to present with earlier symptoms, nevertheless, prognosis is known to be worse, with only 20% to 30% of the patients surviving over a five year period. Methods:A case report of a 47-year-old male who presented with a chronic pigmented lesion on the nipple that was later on diagnosed as PMPD.Results: Microscopic pathological evaluation of mastectomy specimen of the nipple revealed invasive ductal carcinoma Grade II and in situ ductal carcinoma. Estrogen and progesterone receptors were 95% and 80% positive for tumor cells. Atypical cells stained positivite for anticytokeratin 7, c-erbB-2, and CEA on immunohistochemistry while cytoplasmic dusty melanin pigments stained focally positive for S-100 and HMB-45.Conclusion: Although being a rare entity; PMPD should always be kept in mind as a differential diagnosis in melanocytic lesions of the nipple. Histopathology including immunohistochemical staining should always be performed for the correct diagnosis and management.