ABSTRACTPrimary cutaneous adenoid cystic carcinoma (PCACC) is a rare malignant epithelial tumor most commonly observed in the scalp and skin of the chest and originating from the palpebral portion of the lacrimal gland in the orbit. Here we describe the diagnosis and treatment of a rare case of PCACC in an eye of a 52-year-old male. The patient presented with a mass lesion of the right lower eyelid. During incisional biopsy, lack of encapsulation and a secretion pattern different to that of chalazion was observed, which differentiated the lesion from chalazion. Pathological analysis revealed the diagnosis of PCACC. This case highlights the importance of careful inspection for macroscopic differentiation of PCACC from chalazion after initial surgery and pathological evaluation of all surgically removed mass lesions for accurate diagnosis and treatment.
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