| Summary: | Primary cutaneous apocrine carcinoma (PCAC) is an extremely rare malignancy. Distinguishing apocrine carcinoma from breast carcinoma metastasis is difficult. Only a few cases reported as PCAC of the scalp and primarily treated by wide local excision. The usual presentation is a skin lesion that rapidly progresses over the duration of a few weeks to few months. We reported a 56-year-old man with a right scalp ulcerative lesion diagnosed as a CAC. The patient underwent wide local excision. 1.5 years later, the patient developed neck lymph node metastasis that treated with neck dissection and adjuvant radiotherapy. No chemotherapy was given due to limited literature, suggesting substantial benefits of adjuvant chemotherapy for such cases. Review literature was performed to assess the clinical presentation, treatment, and prognosis of such malignancies. PCAC of the scalp is a challenging malignancy in the diagnosis and management.
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