Objective: 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.
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。.
Keywords: Adrenal gland neoplasms; Diagnosis, differential; Immunohistochemistry.