Abstract
Adrenocortical carcinoma (ACC) despite being a rare endocrine malignancy arising from adrenal cortex carries a poor prognosis. It is also challenging to diagnose these tumours preoperatively especially if it is a non-functioning tumour. As the rarity of the disease pose a great challenge in taking up statistically significant studies that would be needed to improve the management strategies and outcomes of ACC, all the recommendations are based on retrospective case series or expert opinions. Hence Multidisciplinary approach is warranted to improve the outcomes in the management of ACC. Despite the enormous progress achieved in the biological knowledge of this tumor, the ACC remains an oncological disease burdened by a high mortality. Surgery is still the first therapeutic option and the only potentially curative treatment.
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