Adrenocortical carcinoma is a rare entity affecting 1–2 people per million. It has a relatively poor prognosis, with an overall 5-year survival of 20–45%. The reasons include a high risk of recurrence following resection and relatively poor response to cytotoxic treatment. The use of the adrenalytic mitotane as adjuvant therapy is supported by level III evidence from cohort studies. In the metastatic setting, one randomized controlled trial provides level II evidence for a tadjuvanthree-drug chemotherapy combination of cisplatin, etoposide and doxorubicin, with mitotane (EDP–M). We present two cases of adrenocortical carcinoma and discuss their management in light of the literature supporting the treatments. These cases illustrate the practicalities of management requiring a multidisciplinary team with the oncologist, endocrinologist, surgeon, anesthetist, radiologist and pathologist, all working in collaboration.
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