Background: Locally advanced tumors account for approximately 50% of children and adolescents with adrenocortical carcinoma (ACC), and of these, up to 50% relapse. We explored the five-item microscopic score and the pS-GRAS score for guiding management. Methods: Data from children and adolescents with COG stage II and III ACC registered in the MET studies were included. The five-item and pS-GRAS score were retrospectively calculated. Results: By December 2021, 55 patients with stage II and III (stage IIn= 18, stage IIIn= 37) had been reported. Median age was 4.3 years [0.1–17.8], median duration of follow-up 6.0 years [0–16.7]. 3-year event-free survival (EFS) rate was 76.5% and 49.8% (p= 0.088), respectively. In stage II tumors, neither the five-item score (p= 0.872) nor pS-GRAS grouping (p= 0.218) had any effect as prognostic factors. In stage III patients, EFS was impaired in tumors with unfavorable histology according to the five-item score (100% vs. 30.8%,p= 0.018). No difference was observed for pS-GRAS groups (p= 0.798). Conclusions: In patients with COG stage III, but not stage II, the five-item score affected EFS. Further studies are needed to identify patients at risk in COG stage II.
背景：局部晚期肿瘤约占儿童及青少年肾上腺皮质癌（ACC）病例的50%，其中高达50%的患者会出现复发。本研究探讨了五项显微镜评分与pS-GRAS评分在指导临床管理中的应用价值。方法：纳入MET研究中登记的儿童及青少年COG分期II期和III期ACC患者数据，回顾性计算五项评分与pS-GRAS评分。结果：截至2021年12月，共报告55例II期和III期患者（II期n=18，III期n=37）。中位年龄4.3岁[0.1-17.8]，中位随访时间6.0年[0-16.7]。II期与III期患者的3年无事件生存率（EFS）分别为76.5%和49.8%（p=0.088）。在II期肿瘤中，五项评分（p=0.872）与pS-GRAS分组（p=0.218）均未显示预后预测价值。在III期患者中，根据五项评分显示组织学不良的肿瘤EFS显著降低（100% vs. 30.8%，p=0.018），而pS-GRAS分组间未观察到差异（p=0.798）。结论：在COG III期（而非II期）患者中，五项评分对EFS具有显著影响。未来需进一步研究以识别COG II期患者中的高危人群。
肿瘤(癌症)患者之家