Kwok Grace T Y, Zhao Jing-Ting, Glover Anthony R, Ip Julian C Y, Sywak Mark, Clifton-Bligh Roderick, Clarke Stephen, Robinson Bruce, Sidhu Stan B
Cancer Genetics Laboratory, Kolling Institute, Northern Sydney Local Health District, Sydney, New South Wales, Australia.
Faculty of Medicine and Health, Royal North Shore Hospital, The University of Sydney, Sydney, New South Wales, Australia.
ANZ J Surg. 2019 Jan;89(1-2):48-52. doi: 10.1111/ans.15032. Epub 2019 Feb 1.
Adrenocortical carcinoma is a rare and heterogeneous malignancy with poor outcomes. Recent research has suggested that outcomes may be improved by centralization of care in specialist centres. We review our evolving 21-year experience in managing adrenocortical carcinoma with a view towards outcomes and lessons learnt.
A retrospective study of patients treated in our specialist endocrine surgical unit over 21 years was undertaken.
Thirty-five patients were treated from diagnosis, 29 forming a primary study cohort. Additionally, seven patients were referred to us for quaternary care, forming a secondary study cohort. The European Network for the Study of Adrenal Tumours (ENSAT) stage and immunohistochemical marker Ki-67 index were strong prognostic indicators for survival.
Early stage, complete resection and Ki-67 <10% are the best prognosticators for survival. Aggressive surgical resection at index operation and of recurrent oligometastatic disease along with multimodal adjuvant treatment has led to long-term survivors of patients with Stage 4 disease in our aggregate cohort.
肾上腺皮质癌是一种罕见的异质性恶性肿瘤，预后较差。最近的研究表明，在专科中心集中治疗可能会改善预后。我们回顾了我们在管理肾上腺皮质癌方面21年不断发展的经验，以期了解治疗结果和汲取的经验教训。
对我们专科内分泌外科病房21年来治疗的患者进行回顾性研究。
从诊断开始共治疗了35例患者，其中29例组成了主要研究队列。另外，有7例患者因接受四级护理被转诊至我们科室，组成了次要研究队列。欧洲肾上腺肿瘤研究网络（ENSAT）分期和免疫组化标志物Ki-67指数是生存的强有力预后指标。
早期、完整切除以及Ki-67<10%是生存的最佳预后因素。在初次手术时积极进行手术切除以及对复发性寡转移疾病进行切除，并结合多模式辅助治疗，使得我们总体队列中4期疾病患者有了长期生存者。
