PDF(Pubmed)
Abstract：
Metastatic adrenocortical carcinoma (ACC) often has a poor outcome, with a five-year survival of less than 25%. We report a rare case of metastatic ACC with a myxoid variant with chromothripsis. We review the histologic variants of ACC, including myxoid type, molecular drivers, and current and investigational therapies for adrenocortical carcinoma. We also discuss the mechanism of chromothripsis, chromothripsis in ACC tumorigenesis, and propose potential therapies targeting chromothripsis.
摘要：
转移性肾上腺皮质癌（ACC）通常预后较差，5年生存率低于25%。我们报告了一例罕见的转移性ACC病例，其粘液样变体带有色素沉着。我们回顾了ACC的组织学变异，包括粘液样类型，分子驱动,以及肾上腺皮质癌的当前和研究疗法。我们还讨论了色素沉着的机制，ACC肿瘤发生中的染色体异常，并提出了针对嗜血杆菌的潜在疗法。
