Nipple Melanoma: A Case Report and Review of the Literature.

Introduction: Primary melanoma of the nipple-areolar complex (NAC) is an exceptionally rare malignancy and may pose significant diagnostic challenges because of its overlap with other breast and cutaneous neoplasms. We report a case of primary NAC melanoma initially misdiagnosed as an undifferentiated sarcoma. Case Presentation: A 93-year-old woman presented with an 8-month history of a bleeding, ulcerated, exophytic lesion of the left nipple. Imaging demonstrated a suspicious NAC mass with radiologically enlarged ipsilateral axillary lymph nodes, while fine-needle aspiration was negative. Core biopsy suggested an undifferentiated sarcoma. Following modified radical mastectomy and axillary lymph node dissection, histopathological and immunohistochemical examination established the diagnosis of ulcerated invasive superficial spreading melanoma of the NAC (Breslow ≥60 mm, pT4bN0M0, and AJCC stage IIC). All 15 lymph nodes were negative. No adjuvant treatment was administered, and the patient remains disease-free 26 months after diagnosis. Conclusion: NAC melanoma should be considered in the differential diagnosis of persistent nipple lesions. Extensive histopathological assessment and comprehensive immunohistochemical studies are essential, particularly in poorly differentiated tumors with heterogeneous marker expression. Early recognition and multidisciplinary management may help avoid diagnostic delays and inappropriate treatment.