摘要目的 探讨黏液样肾上腺皮质腺瘤的临床病理学特征、免疫表型、诊断及鉴别诊断.方法 回顾性分析广东省人民医院2014年1月至2016年12月收集的4例黏液样肾上腺皮质腺瘤的临床资料、病理形态学特征及免疫表型特点,并复习相关文献.结果 4例黏液样肾上腺皮质腺瘤患者26～45岁,其中女性1例,男性3例.肿瘤间质显著的黏液样变性,瘤细胞体积小,核圆形,胞质少-中等量,嗜酸性或透亮,呈条索状、腺管状或器官样排列;其中2例黏液区域占90%以上,1例占70%;而另一黏液区域为10%的病例,肿瘤形成广泛的腺管状结构.免疫组织化学显示4例波形蛋白与Melan A弥漫强阳性,局灶α-抑制素阳性;2例突触素阳性;1例CAM5.2阳性;4例嗜铬粒素A、S-100蛋白、上皮抗原、细胞角蛋白( CK) 7、CK20及广谱细胞角蛋白均阴性.结论 黏液样肾上腺皮质腺瘤非常罕见,是一种良性肿瘤,应避免误诊为其他肾上腺原发或继发的恶性肿瘤,防止造成不适当的治疗.

abstractsObjective To study the clinicopathologic characteristics, immunophenotype, pathologic diagnosis and differential diagnosis of myxoid adrenocortical adenomas. Methods The clinical data, histological features and immunohistochemical results of 4 cases of myxoid adrenocortical adenomas were analyzed, which were collected from January 2014 to December 2016 at Guangdong General Hospital,with review of literature. Results Four cases of myxoid adrenocortical adenomas were presented. The patients ages ranged from 26 to 45 years ( mean ＝35 years). Microscopically, it showed a typical morphology, characterized by small-sized tumor cell cords or pseudo-glands embedded in an abundant extracellular myxoid matrix. Immunohistochemical staining showed tumor cells were strongly positive for Melan A, vimentin and focally for α-inhibin, one case showed strong and diffuse positivity for CAM5.2,and two cases showed diffuse positivity for synaptophysin, while negative for CgA, S-100 protein, epithelial antigen, CK7, CK20 and CKpan.Conclusions Myxoid adrenocortical adenomas are extremely rare,which may cause confusion with metastatic well-differentiated neuroendocrine tumours, sex cord-stromal tumoursor metanephric adenoma. Recognition of this entity would be beneficial for pathologists to avoid misdiagnosis, and unnecessary treatment.