Varghese Jeena, Habra Mouhammed Amir
Department of Endocrine Neoplasia and Hormonal Disorders, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA.
Curr Opin Endocrinol Diabetes Obes. 2017 Jun;24(3):208-214. doi: 10.1097/MED.0000000000000332.
To present an update on the management of and future directions in adrenocortical carcinoma (ACC).
ACC is a rare malignancy with high morbidity and mortality. Surgery remains the mainstay treatment for localized disease, but it is often not feasible in more advanced cases. There is an ongoing controversy about the routine use of adjuvant treatments after surgery. Hormonal overproduction can complicate the management and worsen the prognosis of the disease. Systemic therapy with multiple cytotoxic drugs is often combined with the adrenolytic agent mitotane. Genomic analyses of ACC revealed numerous signal transduction pathway aberrations (insulin-like growth factor 2 overexpression, TP53 mutations and Wnt/β-catenin pathway activation), but so far, there has been no clinically meaningful breakthrough in targeting these genes. Immunotherapy offers hope for altering the orthodox management of cancer, and its role in ACC is being explored in multiple ongoing trials.
Surgery by experienced team is the key treatment for localized ACC, whereas currently used chemotherapy has limited efficacy in advanced ACC. The improved understanding of the molecular pathways involved in ACC has not been translated into effective therapy. The development of new therapies requires collaborative effort to fight this disease.
介绍肾上腺皮质癌（ACC）管理方面的最新进展及未来方向。
ACC是一种罕见的恶性肿瘤，发病率和死亡率都很高。手术仍然是局限性疾病的主要治疗方法，但在病情更晚期的情况下往往不可行。关于术后辅助治疗的常规使用存在争议。激素过度分泌会使疾病管理复杂化并恶化预后。多种细胞毒性药物的全身治疗通常与溶肾上腺药物米托坦联合使用。ACC的基因组分析揭示了许多信号转导通路异常（胰岛素样生长因子2过表达、TP53突变和Wnt/β-连环蛋白通路激活），但到目前为止，针对这些基因尚未取得具有临床意义的突破。免疫疗法为改变癌症的传统管理带来了希望，其在ACC中的作用正在多项正在进行的试验中进行探索。
由经验丰富的团队进行手术是局限性ACC的关键治疗方法，而目前使用的化疗在晚期ACC中的疗效有限。对ACC所涉及分子通路的更好理解尚未转化为有效的治疗方法。新疗法的开发需要共同努力来对抗这种疾病。
