儿童和青少年局部晚期肾上腺皮质癌——疑难且具有挑战性的病例

Locally Advanced Adrenocortical Carcinoma in Children and Adolescents-Enigmatic and Challenging Cases.

作者信息

Kuhlen Michaela, Mier Pascal, Kunstreich Marina, Lessel Lienhard, Slavetinsky Christoph, Fuchs Jörg, Seitz Guido, Holterhus Paul-Martin, Wudy Stefan A, Vokuhl Christian, Frühwald Michael C, Vorwerk Peter, Redlich Antje

机构信息

Pediatrics and Adolescents Medicine, Faculty of Medicine, University of Augsburg, 86156 Augsburg, Germany.

Pediatric Hematology/Oncology, Department of Pediatrics, Otto von Guericke University Children's Hospital, 39106 Magdeburg, Germany.

出版信息

Cancers (Basel). 2023 Aug 28;15(17):4296. doi: 10.3390/cancers15174296.

DOI:10.3390/cancers15174296

PMID:37686571

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10486626/

Abstract

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 II = 18, stage III = 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% ( = 0.088), respectively. In stage II tumors, neither the five-item score ( = 0.872) nor pS-GRAS grouping ( = 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%, = 0.018). No difference was observed for pS-GRAS groups ( = 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.

摘要

背景

局部晚期肿瘤约占儿童和青少年肾上腺皮质癌（ACC）患者的50%，其中高达50%会复发。我们探索了五项微观评分和pS-GRAS评分以指导治疗。

方法

纳入在MET研究中登记的COG II期和III期ACC儿童和青少年的数据。回顾性计算五项评分和pS-GRAS评分。

结果

截至2021年12月，已报告55例II期和III期患者（II期 = 18例，III期 = 37例）。中位年龄为4.3岁[0.1 - 17.8]，中位随访时间为6.0年[0 - 16.7]。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）和pS-GRAS分组（P = 0.218）均未作为预后因素产生任何影响。在III期患者中，根据五项评分，组织学不良的肿瘤EFS受损（100%对30.8%，P = 0.018）。pS-GRAS组间未观察到差异（P = 0.798）。