Abstract
Adrenocortical Carcinoma (ACC) is a rare but aggressive malignancy with poor prognosis and limited response to available systemic therapies. Although complete surgical resection gives the best chance for long-term survival, ACC has a two-year recurrence rate of 50%, which poses a therapeutic challenge. High throughput analyses focused on characterizing the molecular signature of ACC have revealed specific micro-RNAs (miRNAs) that are associated with aggressive tumor phenotypes. MiRNAs are small non-coding RNA molecules that regulate gene expression by inhibiting mRNA translation or degrading mRNA transcripts and have been generally implicated in carcinogenesis. This review summarizes the current insights into dysregulated miRNAs in ACC tumorigenesis, their known functions, and specific targetomes. In addition, we explore the possibility of particular miRNAs to be exploited as clinical biomarkers in ACC and as potential therapeutics.
Highlights
Adrenal tumors are very common, affecting up to 10% of the general population, of which the large majority are benign non-functional adenomas [1]
Adrenocortical cancer (ACC) was associated with loss of heterozygosity (LOH) at the Tumor Suppressor Protein 53 (TP53) gene locus 17p13.1 in 74% of cases compared with only 14% of adrenal adenomas [15]
ACC affects approximately seven percent of children with Beckwith–Weidemann Syndrome (BWS) [16], which is caused by mutations or epigenetic modifications at the genetic locus 11p15 containing the Insulin-Like Growth Factor 2 (IGF2) gene. 11p15
Summary
Adrenal tumors are very common, affecting up to 10% of the general population, of which the large majority are benign non-functional adenomas [1]. Cancers 2020, 12, 2198 chemotherapy-based phase III clinical trial for advanced ACC (First International Randomized Trial in Locally Advanced and Metastatic Adrenocortical Carcinoma Treatment (FIRM-ACT)) was completed in. ACC research is currently focused on developing methods for early detection and effective management of a metastatic disease. Over the past two decades, miRNA research in cancer has focused on determining the miRNA expression signatures of different tumors in order to identify potential biomarkers for early diagnosis, as well as functional studies of specific miRNAs to determine their targets and function. MiRNAs have been identified to have both diagnostic and therapeutic potential in the cancer literature, broadening our understanding of their roles in tumor biology. We present a current summary of the mounting body of work describing miRNA dysfunction in ACC with the aims of highlighting their potential function and roles in modulating key oncogenic pathways
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