摘要目的 探讨微囊肿附属器癌(MAC)的诊断及鉴别诊断.方法 回顾分析2003-2017年中国医学科学院皮肤病医院诊治的10例MAC的临床、病理和免疫组化特点、治疗和预后.结果 10例患者中男3例,女7例,平均发病年龄51.65岁.皮损均位于面部,6例位于上唇部,表现为单发斑块或结节,形成溃疡4例.组织病理均由上皮细胞条索组成,可见不同数量的角囊肿和导管结构,6例见神经受累,核分裂象少见.免疫组化示上皮细胞及角囊肿角蛋白阳性,导管及腺腔结构癌胚抗原及上皮膜抗原阳性.所有患者均接受手术切除治疗,1例13年后原位复发,均无远处转移.结论 MAC皮损主要表现为上唇的红斑块,部分破溃,确诊依赖于向毛囊和汗腺双相分化的特征性组织病理学改变,免疫组化有助于与其他附属器肿瘤相鉴别.

abstractsObjective To discuss the diagnosis and differential diagnosis of microcystic adnexal carcinoma (MAC).Methods Totally,10 patients with MAC were enrolled from Hospital for Skin Diseases,Chinese Academy of Medical Sciences from 2003 to 2017.Their clinical manifestations,histopathological and immunohistochemical features,treatment and prognosis were retrospectively analyzed.Results Of the 10 patients,3 were males and 7 were females.Their average age at the onset of MAC was 51.65 years.Skin lesions all occurred on the face,and on the upper lip in 6 cases.The lesions usually presented as solitary plaque or nodule,and ulceration occurred in 4 cases.Histopathologically,skin lesions consisted of epithelial cords with different numbers of keratinous cysts and tubular structures,and neural involvement occurred in 6 cases.However,mitotic figures were rare.Immunohistochemical staining showed epithelial cells and keratinous cysts stained positive for cytokeratin,as well as tubular structures and glandular cavities stained positive for carcinoembryonic antigen and epithelial membrane antigen.All the patients received surgical excision,and one patient experienced in situ recurrence 13 years later.No distant metastasis occurred in these patients.Conclusions MAC mainly presents as red plaques with occasional ulceration on the upper lip.Its definite diagnosis depends on characteristic histopathological changes in bidirectional differentiation into hair follicles and sweat glands,and immunohistochemical features are helpful to distinguish MAC from other adnexal tumors.