腎上腺皮質癌是一種少見癌症，發生率約為每十萬人口0.5到2人。因為初期症狀不明顯，大多為接受影像學檢查時意外發現，且發現時多已屬晚期，預後相當差。約有60％屬於功能性腫瘤，分泌以腎上腺皮質素為主，臨床表現則以庫欣氏症候群為多。研究顯示，腫瘤細胞並無特定免疫組織學染色特徵，也無特定基因表現型，故在治療上相當棘手。目前公認最好的治療方式為手術切除，不只是有潛在治癒的可能，在已轉移的病人身上也有改善症狀及延長存活期的報告。腫瘤對化學治療反應不佳，而放射線治療則有部份報告指出可以局部控制疾病，降低局部復發的風險；Mitotane常用於手術後輔助治療、或無法手術者第一線治療、甚至和其他化學治療合併使用，在目前使用報告中，均能有效延後病人復發的時間，也可以增加五年存活率。

Adrenocortical carcinoma is a rare cancer; its incidence is about 0.5 to 2 per 1000000 people. Because of initial symptoms are not significance, most of them were diagnosed by the image study incidentally, and it has the poor prognosis due to the late stage of the tumor at diagnostic time. About 60％ of the cases were functional tumor, mainly secreted the corticosteroid, which presented as Cushing’s syndrome. There was no specific feature of immunochemical stain of the tumor, as no specific genotyping, and it made the difficulty to the treatment. The best treatment now is the surgical resection; it may cure the disease potentially and also could relief the symptoms and increase survival period of the metastatic disease. It has the poor response to chemotherapy, but some reports showed the radiotherapy has the benefit for control the local disease, which decreased the risk of local recurrence. Mitotane is used as adjuvant therapy after the surgery, the first line therapy for the unresectable disease, or combination therapy with the chemotherapy. In those studies, it could delay the time of recurrence, and it also could increase the five year survival rate.