Dept of Pathology, MD Anderson Cancer Center, 1515 Holcombe Blvd, Houston, TX 77030, USA.
Am J Clin Pathol. 2013 Jun;139(6):780-6. doi: 10.1309/AJCPCDZLC13RSXRZ.
Adrenocortical carcinomas (ACCs) with myxoid features are rare neoplasms. We identified 7 cases of myxoid ACC and studied the clinicopathologic and immunohistochemical features of these neoplasms. The patients were 5 men and 2 women with a mean age of 45 years. Histologically, the tumors contained alcian blue-positive myxoid areas ranging from 10% to 50% of the tissue examined. One case showed lipomatous metaplasia. Areas of conventional ACC were present in all cases. Immunohistochemically, the tumors were positive for steroid receptor cofactor 1, inhibin, melan A, calretinin, and synaptophysin but negative for high-molecular-weight cytokeratin, CAM5.2, and Pax8. Clinical follow-up information for 4 patients demonstrated that all patients had died of their disease 11 to 69 months after diagnosis. Myxoid ACCs are rare tumors that expand the differential diagnosis of myxoid neoplasms involving the retroperitoneum. Contrary to previous reports proposing that the biologic behavior is similar to conventional ACC, our series seems to indicate that myxoid morphology is associated with more aggressive behavior.
具有黏液样特征的肾上腺皮质癌 (ACC) 是罕见的肿瘤。我们鉴定了 7 例黏液样 ACC，并研究了这些肿瘤的临床病理和免疫组织化学特征。患者为 5 男 2 女，平均年龄 45 岁。组织学上，肿瘤含有范围为 10%至 50%的组织学检查的阿尔辛蓝阳性黏液样区域。1 例显示脂肪化生。所有病例均存在常规 ACC 区域。免疫组织化学染色，肿瘤阳性表达类固醇受体辅因子 1、抑制素、黑素 A、钙视网膜蛋白和突触素，但阴性表达高分子量细胞角蛋白、CAM5.2 和 Pax8。对 4 例患者的临床随访信息显示，所有患者在诊断后 11 至 69 个月内均因疾病死亡。黏液样 ACC 是罕见肿瘤，扩大了涉及腹膜后黏液样肿瘤的鉴别诊断。与先前提出的生物学行为类似于常规 ACC 的报告相反，我们的研究似乎表明黏液样形态与更具侵袭性的行为相关。
