Internal Medicine, Department of Clinical and Biological Sciences, S. Luigi Hospital, Orbassano, University of Turin, Turin, Italy.
Division of Endocrinology and Diabetes, Department of Internal Medicine, University Hospital, University of Würzburg, Würzburg, Germany.
Eur J Endocrinol. 2022 Jul 19;187(3):R27-R40. doi: 10.1530/EJE-22-0260. Print 2022 Sep 1.
Adrenocortical carcinoma (ACC) accounts for a minority of all malignant tumors in adults. Surgery remains the most important therapeutic option for non-metastatic ACC. Whether a subset of patients with small ACC may benefit from minimally invasive surgery remains a debated issue, but we believe that surgeon's expertise is more important than surgical technique to determine outcome. However, even a state-of-the-art surgery cannot prevent disease recurrence that is determined mainly by specific tumor characteristics. We consider that the concomitant presence of the following features characterizes a cohort of patients at low risk of recurrence, (i) R0 resection (microscopically free margin), (ii) localized disease (stage I-II ACC), and (iii) low-grade tumor (ki-67 <10%). After the ADIUVO study, we do not recommend adjuvant mitotane as a routine measure for such patients, who can be managed with active surveillance thus sparing a toxic treatment. Patients at average risk of recurrence should be treated with adjuvant mitotane. For patients at very high risk of recurrence, defined as the presence of at least one of the following: Ki67 >30%, large venous tumor thrombus, R1 resection or stage IV ACC, we increasingly recommend to combine mitotane with four cycles of platinum-based chemotherapy. However, patients at moderate-to-high risk of recurrence should be ideally enrolled in the ongoing ADIUVO2 trial. We do not use adjuvant radiotherapy of the tumor bed frequently at our institutions, and we select patients with incomplete resection, either microscopically or macroscopically, for this treatment. In the long-term, prospective multicenter trials are required to improve patient care.
肾上腺皮质癌（ACC）在成年人所有恶性肿瘤中占少数。手术仍然是非转移性 ACC 的最重要治疗选择。一小部分小 ACC 患者是否可能受益于微创手术仍然是一个有争议的问题，但我们认为外科医生的专业知识比手术技术更重要，以确定结果。然而，即使是最先进的手术也不能预防主要由特定肿瘤特征决定的疾病复发。我们认为，以下特征的共同存在可以确定一组复发风险低的患者，（i）RO 切除（显微镜下无边缘），（ii）局限性疾病（I 期-II 期 ACC），和（iii）低级别肿瘤（ki-67<10%）。在 ADIUVO 研究之后，我们不建议对这些患者常规使用辅助米托坦，他们可以通过积极监测来管理，从而避免毒性治疗。具有平均复发风险的患者应接受辅助米托坦治疗。对于复发风险非常高的患者，定义为存在以下至少一项：Ki67>30%，大静脉肿瘤血栓，R1 切除或 IV 期 ACC，我们越来越建议将米托坦与四个周期的铂类化疗相结合。然而，具有中至高复发风险的患者应理想地参加正在进行的 ADIUVO2 试验。我们在机构中不经常使用肿瘤床辅助放疗，我们为这种治疗选择显微镜下或肉眼下有不完全切除的患者。在长期，需要进行前瞻性多中心试验来改善患者护理。
