Adrenal cortical carcinoma (ACC) is a rare malignant tumour which arises from the adrenal cortex with diverse clinical manifestations due to excessive hormone production, with Cushing’s syndrome and virilisation being the most common features. The diagnosis of ACC relies on clinical, hormonal, and imaging features before surgery and pathological examination after tumour removal. Pathological assessment of Weiss score and the Ki-67 labelling index play an important role in diagnosis and prognosis. The tumour, lymph node, and metastasis (TNM) classification, established by the European Network for the Study of Adrenal Tumors (ENSAT) is used for staging. Currently, complete surgical resection is the only treatment option for ACC that has a curative intent, with no reported difference in overall recurrence or mortality between open and laparoscopic adrenalectomy. Mitotane is used as an adjuvant therapy after surgery for high-risk patients and as primary treatment for unresectable and advanced cases; however, it has a narrow therapeutic index and regular blood monitoring is essential. The role of chemotherapy and radiotherapy in ACC is unclear with limited studies and varying results. To date, trials which have been conducted for novel agents revealed disappointing results. Currently, trials are underway for agents targeting steroidogenic factor-1, mechanistic target of rapamycin (mTOR), and Wnt signalling pathways, as well as inhibitors of acetyl-coA cholesterol acetyltransferase 1. Unfortunately, ACC has an aggressive natural course with high recurrence rate, and a reported 10-year survival of 7% after treatment.
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