目的:观察皮肤腺样囊腺癌的病理形态及免疫学特点,并探讨其鉴别诊断和组织来源。方法:对1例皮肤腺样囊腺癌进行光镜及免疫组化染色,结合临床病理、免疫组化等特点进行探讨。结果:病理上为单一形态的基底样细胞,瘤细胞呈巢、索状分化成大小不一、内含丰富黏液的恶性肿瘤,免疫组化CEA阳性,CK弱阳性,EM A阴性。结论:皮肤腺样囊腺癌具有较复杂的组织学特点,必须依靠病理组织活检才能作出正确诊断。 Objective: To observe the pathological features and immunological characteristics of dermoid adenoid cystadenocarcinoma and to explore its differential diagnosis and tissue source. Methods: One case of adenoid cystadenocarcinoma was examined by light microscopy and immunohistochemistry, combined with clinicopathological features and immunohistochemistry. Results: The single pathological basaloid cells were neoplasm in neoplasm. The neoplasm differentiated into malignant tumors of various sizes and mucus. Immunohistochemical staining for CEA, weakly positive CK and negative EM A were found. Conclusion: Adenoid cystadenocarcinoma of the skin has more complicated histological features and must be relied on biopsy of pathological tissue to make a correct diagnosis.