Background: Mammary Paget’s disease (MPD) accounts for 1—4% of all breast carcinomas. It can affect both men and women with a wide age range, 27 to 88 years old, and 20—30% affect premenopausal woman. Moreover, no specific risk factor has been identified for MPD, and the exact pathogenesis remains uncertain. For these reasons, the MPD clinicopathologically engages in case reports. Case Presentation: A 46-year-old woman with a palpable mass in the left breast for 6 months. The ultrasound examination showed hypervascularity accompanied by nipple abrasions, and the mammography results did not identify malignant calcification or mass formation. Routine histopathology revealed anaplastic cells with round cell morphology, and partially clear, extensive eosinophilic cytoplasm was seen. In addition, immunohistochemical examination showed estrogen receptor (ER), progesterone receptor (PR), and human epidermal growth factor receptor 2 (HER2) strongly positive with 60% Ki67 proliferation index. Conclusions: Based on clinical data, radiological, combined with histopathological and immunohistochemical examination, the case supports mammary Paget disease with ductal carcinoma in situ (DCIS). It has an excellent outcome, > 95% survival at 20 years
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