Adrenocortical carcinoma (ACC) is a rare malignancy that is frequently asymptomatic at presentation, yet has a high rate of metastatic disease at the time of diagnosis. Prognosis is overall poor, particularly with cortisol-producing tumors. While the treatment of ACC is guided by stage of disease, complete surgical resection is the most important step in the management of patients with primary, recurrent, or metastatic ACC. Triphasic chest, abdomen, and pelvis computer tomography (CT) scans and 18F flourodeoxyglucose positron emission tomography CT scanning are essential for accurate staging; moreover, MRI may be helpful to identify liver metastasis and evaluate the involvement of adjacent organs for operative planning. Surgical resection with negative margins is the single most important prognostic factor for survival in patients with ACC. To achieve the highest rate of R0 resection, open adrenalectomy is the gold standard surgical approach for confirmed or highly suspected ACC. It is extremely important that the tumor capsule is not ruptured, regardless of the surgical approach used. The best post-operative outcomes (complications and oncologic) are achieved by high-volume surgeons practicing at high-volume centers.

肾上腺皮质癌（ACC）是一种罕见的恶性肿瘤，表现时通常无症状，但在诊断时具有很高的转移性疾病发生率。预后总体较差，特别是皮质醇产生的肿瘤。尽管ACC的治疗取决于疾病的阶段，但完整的手术切除是治疗原发性，复发性或转移性ACC患者最重要的步骤。三阶段性胸部，腹部和骨盆计算机断层扫描（CT）扫描和18F脱氧葡萄糖葡萄糖正电子发射断层扫描CT扫描对于准确分期至关重要。此外，MRI可能有助于识别肝转移并评估邻近器官的参与以进行手术计划。切缘阴性的手术切除是ACC患者生存的最重要的预后因素。为了达到最高的R0切除率，开放性肾上腺切除术是确诊或高度怀疑的ACC的金标准手术方法。无论使用哪种手术方法，肿瘤囊都不会破裂是非常重要的。最好的术后结果（并发症和肿瘤学）是由在高容量中心执业的高容量外科医生实现的。