【摘要】 目的:探讨伴有假腺样结构的黏液样肾上腺皮质腺瘤(myxoid adrenocortical adenomas, MACA)的临床病理学特点和生物学行为。方法:回顾性分析我院1例伴有假腺样结构的MACA的临床病理学特征，采用免疫组化方法检测肿瘤组织Vimentin、Melan-A、CD56、NSE等标记的表达，并进行随访和相关文献复习。结果:患者男性，年龄49岁，体检发现右肾上腺占位。肿块表面光滑，切面淡黄色或灰白色，实性，局部区域呈胶冻样改变。镜下，肿瘤大部被覆薄层纤维性包膜，周围可见少量肾上腺组织；局灶区域肿瘤细胞累犯纤维包膜和周围脂肪组织。肿瘤特征性富含黏液性间质背景，肿瘤细胞排列呈腺样、微囊状、梁索状及肾小球样，瘤细胞形态大小一致，中等偏小，胞质略嗜酸或透明，核圆形至卵圆形，核分裂象罕见，未见坏死及脉管侵犯。免疫组织化学显示瘤细胞阳性表达Vimentin、Melan-A、CD56、NSE,Ki-67增殖指数小于5%;特殊染色显示黏液样物质阿尔辛蓝阳性。结论:MACA是一种非常罕见的肾上腺皮质良性肿瘤，常伴有假腺样结构，但包膜累犯较少见，由于病例少，这种生长方式有无临床病理意义，需待进一步观察。临床上诊断该肿瘤时，需综合临床特征、影像学及病理特征，同时除外转移性腺癌及其他伴有黏液变性的腹膜后肿瘤，完整切除并进行随访。更多还原

【Abstract】 Objective:To study the clinicopathological characteristics and the behavior of pseudoglandular myxoid adrenocortical adenoma(MACA).Methods:A case of MACA with pseudoadenoid structure was reviewed.The expression of Vimentin, Melan-A,CD56,NSE in tumor tissue was detected by immunohistochemistry.Meanwhile follow up and literature review were performed.Results:A 49-year-old male patient presented with a right adrenal gland tumor.Grossly, the mass was mostly solid, roundish and with clear boundaries, cutting indicated greyish white to local translucence.Histologically, tumor was demarcated by a thin fibrous capsule, focal involvement of fibrous capsule and atrophic adrenal gland tissue were found.The tumor was arranged in pseudoglandular pattern and cords, microcystic, glomerular structure with a rich background of mucus.The tumor cells were small to medium, eosinophilic, or transparent, with round to oval nucleus, occasionally visible nucleoli, and rare mitotic figure.The tumor showed no evidence of necrosis or vascular invasion.Immunohistochemical staining showed positive expression of Vimentin, Melan-A,CD56,NSE,and Ki-67 index was less than 5%.Histochemical staining for Alcian-blue highlighted the abundant eosinophilic myxoid material.Conclusion:MACA is a rare benign tumor of adrenal neoplasm frequently accompanied by a pseudoglandular pattern, but capsule invasion was scarce.Clinicopathologic significance of this growth pattern needs more cases to be further observed.The clinical diagnosis of the tumor should be based on the clinical features,imaging and pathological features,while excluding metastatic adenocarcinoma and other retroperitoneal tumors with mucinous degeneration. The patients should be carefully followed-up after complete excision.更多还原