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Adrenocortical carcinoma has an incidence of 1 per million people and is a rare and aggressive disease. A big tumor's compressive symptoms, hormonal production, or an unintentional finding on imaging tests all contribute to the diagnosis. The best opportunity for a cure is provided via surgery, which has been the basis of treatment. Metastatic disease and local recurrence, however, are frequent. Stage I illness has an 82 percent 5-year survival rate, whereas stage IV disease, which is indicated by distant metastases, has a 13% survival rate. 70% of patients have stage III or stage IV illness when they first arrive. Consequently, a lot of people need systemic treatment. Since 1960, mitotane adjuvant therapy has been the sole authorised treatment for ACC. Patients who have a high risk of recurrence are advised to get adjuvant radiation to the tumour bed. Palliative chemotherapy with etoposide, doxorubicin, and cisplatin with mitotane is the current standard of care if the illness has spread to other organs. Response rates to the EDP + mitotane regimen are 23%. Systemic targeted medicines have had less success than this plan. For metastatic illness, ablative methods are frequently employed. To increase the possibilities for this patient population, further medicines are still being researched, and collaborative organisations have been established all around the world.
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