Adrenocortical carcinoma (ACC) is a rare endocrine malignancy arising from the adrenal cortex often with unexpected biological behavior. It can occur at any age, with two peaks of incidence: in the first and between fifth and seventh decades of life. Although ACC are mostly hormonally active, precursors and metabolites may be also produced by dedifferentiated and immature malignant cells. Distinguishing the etiology of an adrenal mass, between benign adenomas, which are quite frequent in general population, and malignant carcinomas with dismal prognosis is challenging. However, recent advances in genomic, pathology, and staging allow the development of standardization of pathology reporting and refinement of prognostic grouping for planning treatment of the patients with ACC. Besides, no single histopathological as well as no single imaging method, hormonal work-up, or immunohistochemical labelling can definitively prove the diagnosis of ACC. Over several decades’ great efforts have been made in finding novel reliable and available diagnostic and prognostic factors including steroid metabolome profiling or target gene identification. Preliminary data show that for localized ACC, molecular makers (gene expression, methylation, and chromosome alterations) could predict cancer recurrence. Nevertheless, many of these markers need further validation and some are difficult to be widely applied in clinical settings. ACC is frequently diagnosed in advanced stages and therapeutic options are unfortunately limited. Surgery remains the “gold standard’ treatment. The management of patients with ACC requires a multidisciplinary approach. Immunotherapy in advanced ACC has been investigated in different studies however, the reported rates of overall response rate and progression free survival were generally poor. Thus, new biological markers that could predict patient prognosis and provide individualized therapeutic options are required. For complete coverage of all related areas of Endocrinology, please visit our on-line FREE web-text,
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