Libé Rossella
French Network for Adrenal Cancer, Department of Endocrinology, Cochin Hospital, Paris, France -
Minerva Endocrinol. 2019 Mar;44(1):58-69. doi: 10.23736/S0391-1977.18.02900-0. Epub 2018 Sep 12.
Adrenocortical carcinoma (ACC) is a rare cancer, with an incidence less than 0.7-1.5 per 1 million people per year, with a poor prognosis. The overall survival (OS) depends on the ENSAT stage: in particular in metastatic ACC the OS varies from 10 to 20 months, with a 5-year survival around 10%. ACC has a different behavior, probably due to a different biology. For this reason, a careful prognostic classification is mandatory, in order to stratify the patients and propose a specific management.
Prognostic factors can be divides in three groups: clinical factors (tumor stage, age, hormone-related symptoms), pathological factors (Weiss Score, mitotic count, Ki-67, SF-1 and AVA2, P53, beta-catenin immunohistochemistry, resection status), molecular factors (chromosomal aberrations, methylation profile, altered gene expression and miRNA expression, gene mutations).
The best way to stratify ACC patients and propose the best therapeutic option is to combine clinical, pathological and molecular factors.
Individualizing patients' prognosis and tumor biology appears as a necessary step for personalized medicine. In addition to tumor stage and tumor grade, the genomic classification may precise the risk stratification and thus help defining therapeutic strategy.
肾上腺皮质癌（ACC）是一种罕见癌症，年发病率低于百万分之0.7 - 1.5，预后较差。总生存期（OS）取决于欧洲肾上腺肿瘤研究小组（ENSAT）分期：特别是转移性ACC，其OS为10至20个月不等，5年生存率约为10%。ACC具有不同的生物学行为，可能归因于不同的生物学特性。因此，进行细致的预后分类很有必要，以便对患者进行分层并提出具体的治疗方案。
预后因素可分为三组：临床因素（肿瘤分期、年龄、激素相关症状）、病理因素（魏斯评分、有丝分裂计数、Ki-67、SF-1和AVA2、P53、β-连环蛋白免疫组化、切除状态）、分子因素（染色体畸变、甲基化谱、基因表达改变和微小RNA表达、基因突变）。
对ACC患者进行分层并提出最佳治疗方案的最佳方法是综合临床、病理和分子因素。
个体化患者的预后和肿瘤生物学特性似乎是精准医疗的必要步骤。除肿瘤分期和肿瘤分级外，基因组分类可精确风险分层，从而有助于确定治疗策略。
