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Updated: Jul 16, 2025
A Novel Method: Super-selective Adrenal Venous Sampling
Published on: September 15, 2017
Locally Advanced Adrenocortical Carcinoma in Children and Adolescents-Enigmatic and Challenging Cases
Michaela Kuhlen1, Pascal Mier2, Marina Kunstreich1,2
1Pediatrics and Adolescents Medicine, Faculty of Medicine, University of Augsburg, 86156 Augsburg, Germany.
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 n = 18, stage III n = 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.
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