Saeger Wolfgang, Mohren Werner, Behrend Matthias, Iglauer Peter, Wilczak Waldemar
Institute of Pathology and Neuropathology of the University of Hamburg, Martinistraße 52, 20246, Hamburg, UKE, Germany.
Institute of Pathology, Hospital Deggendorf, 94469, Deggendorf, Germany.
Endocr Pathol. 2017 Jun;28(2):139-145. doi: 10.1007/s12022-016-9450-7.
A tumor in the adrenal region with two metastases in the liver was classified as poorly differentiated sarcoma on the base of extensive immunostainings (expression of vimentin, desmin, myogenin, and CD31, no expression of inhibin, melan A). Four years later in a second examination with molecular methods for a study of adrenal sarcomas, this diagnosis must be revised due to the lack of MDM-2 gene amplification and FKHR translocation which exclude sarcoma. Further immunostainings of many other parts of the tumor showed in one area more mature tumor tissue expressing synaptophysin, SF-1, and melan A. From these findings we classified an adrenal cortical cancer with predominant dedifferentiation into a sarcomatoid adrenal carcinoma. The properties of this very rare cancer type are presented and discussed.
肾上腺区域的一个肿瘤，肝脏有两处转移灶，基于广泛的免疫染色（波形蛋白、结蛋白、肌细胞生成素和CD31表达，抑制素、黑素A无表达）被分类为低分化肉瘤。四年后，在第二次检查中采用分子方法研究肾上腺肉瘤时，由于缺乏MDM - 2基因扩增和FKHR易位（排除肉瘤），此诊断必须修正。对肿瘤许多其他部位的进一步免疫染色显示，在一个区域有更成熟的肿瘤组织表达突触素、SF - 1和黑素A。根据这些发现，我们将以去分化为主的肾上腺皮质癌分类为肉瘤样肾上腺皮质癌。本文介绍并讨论了这种非常罕见的癌症类型的特性。
