Contemporary preclinical human models of adrenocortical carcinoma.
Contemporary preclinical human models of adrenocortical carcinoma.
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DOI：
10.1016/j.coemr.2019.08.009
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发表时间：
2019-10-01
影响因子：
--
通讯作者：
Hantel, Constanze
中科院分区：
文献类型：
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作者：
Pinto, Emilia Modolo;Kiseljak-Vassiliades, Katja;Hantel, Constanze
肾上腺皮质癌（ACC）是一种罕见的异质性疾病，在儿童和成人中可能表现不同。ACC的治疗取决于疾病的分期，完全手术切除是唯一可能治愈的治疗方法。第一个和最广泛使用的肾上腺皮质癌细胞系，作为模型系统，以检查控制肾上腺皮质的正常和病理功能的机制，最初是在1980年分离。尽管NCI-H295保持了类固醇的能力和肾上腺皮质的特征，但缺乏新的ACC细胞系和动物模型阻碍了新疗法的进展和开发。在这篇综述中，我们提供了细胞和患者来源的肿瘤异种移植（PDTX）模型ACC产生的基本致病机制和临床前功能研究，这种侵略性疾病的阐明。
Adrenocortical carcinoma (ACC) is an uncommon and heterogeneous disease and may present differently in children and adults. Management of ACC is dependent on disease stage and complete surgical resection is the only potentially curative treatment. The first and most extensively used adrenocortical cancer cell line, as model system to examine mechanisms controlling normal and pathologic function of adrenal cortex, was initially isolated in 1980. Although NCI-H295 maintained steroid capabilities and adrenocortical characteristics, the lack of new cell lines and animal models of ACC has hampered the progress and development of new therapies. In this review we provide description of cellular and patient-derived tumor xenograft (PDTX) models of ACC generated for the elucidation of the underlying pathogenic mechanisms and preclinical functional studies for this aggressive disease.