Erickson Lori A, Rivera Michael, Zhang Jun
Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN.
Adv Anat Pathol. 2014 May;21(3):151-9. doi: 10.1097/PAP.0000000000000019.
Adrenocortical carcinoma is a rare endocrine tumor with a poor prognosis. These tumors can be diagnostically challenging, and diagnostic algorithms and criteria continue to be suggested. Myxoid and oncocytic variants are important to recognize to not confuse with other tumors. In addition, the diagnostic criteria are different for oncocytic adrenal carcinomas than conventional carcinomas. Adrenocortical carcinomas usually occur in adults, but can also occur in children. In children these tumors are diagnostically challenging as the histologic features of malignancy seen in an adult tumor may not be associated with aggressive disease in a child. Adrenocortical carcinomas occur with increased frequency in Beckwith-Wiedemann and Li-Fraumeni syndromes, but most occur sporadically. Gene expression profiling by transcriptome analysis can discriminate adrenocortical carcinomas from adenomas and divide carcinomas into prognostic groups. The increasing understanding of the pathogenesis of these tumors may provide increasing treatment targets for this aggressive tumor.
肾上腺皮质癌是一种罕见的内分泌肿瘤，预后较差。这些肿瘤在诊断上具有挑战性，诊断算法和标准仍在不断被提出。黏液样和嗜酸细胞样变体对于识别很重要，以免与其他肿瘤混淆。此外，嗜酸细胞性肾上腺皮质癌的诊断标准与传统癌不同。肾上腺皮质癌通常发生于成年人，但也可发生于儿童。在儿童中，这些肿瘤在诊断上具有挑战性，因为在成人肿瘤中所见的恶性组织学特征可能与儿童侵袭性疾病无关。肾上腺皮质癌在贝克威思-维德曼综合征和李-弗劳梅尼综合征中发病率增加，但大多数为散发性。通过转录组分析进行基因表达谱分析可以区分肾上腺皮质癌和腺瘤，并将癌分为不同的预后组。对这些肿瘤发病机制的日益了解可能为这种侵袭性肿瘤提供越来越多的治疗靶点。
