Adrenocortical Carcinoma

Adrenal cortical carcinoma (ACC) is an uncommon malignant tumor that arises from the adrenal cortex and leads to various clinical manifestations because of excessive hormone production and local extension. Clinical, hormonal, radiological, and histopathological examinations diagnose ACC. Pathological assessment using the Weiss score and the Ki-67 labeling index is crucial in diagnosing and prognosis. Complete surgical resection is the only curative option for treating ACC. In high-risk patients, post-surgery adjuvant mitotane is recommended. Mitotane can also be considered a primary treatment in unresectable and advanced cases, primarily in secretary ACC. Palliative chemotherapy is indicated in advanced or unresectable locally advanced cases. The role of radiotherapy in ACC is still evolving. Trials of various newer therapies targeting the steroidogenic factor-1, mechanistic target of rapamycin (mTOR), Wnt signaling pathways, and acetyl-CoA are underway. The outcome of the ACC is still poor, despite promising advances in molecular characterization and understanding of the ways of tumorigenesis.