Endocrine oncology

Assessment of prognostic factors in pediatric adrenocortical tumors: the modified pediatric S-GRAS score in an international multicenter cohort—a work from the ENSAT-PACT working group

Eur J EndocrinolPublished Multicenter retrospective cohort study

Authors' conclusion

In an international multicenter study, we confirmed that the pS-GRAS score is strongly associated with overall survival among patients with pACC. Age, resection status, stage, and Ki67 index are important parameters for risk stratification.

Study context

Population
Children with pediatric ACC (pediatric adrenocortical carcinoma (pACC)) from 9 centers in 6 countries (ENSAT-PACT, GPOH-MET, IC-PACT); median age 4 years
Participants
268 children
Exposure
pS-GRAS score groups 1–4
Outcome
Overall survival (OS)
Methodological detailsprognostic score validation, international, 9 centers, 6 countries
  • Prognostic score validation
  • International
  • 9 centers, 6 countries

Main finding

As reported in the abstract

OS by pS-GRAS group · 5-year
GroupValueHR of death
Group 198%
Group 287%3.6 (95% CI 1.6-8.2)
Group 343%2.8 (95% CI 1.9-4.4)
Group 418%2.1 (95% CI 1.7-2.7)

Value

Group 198%
Group 287%
Group 343%
Group 418%

HR of death

Group 1— not stated in the abstract
Group 23.6 (95% CI 1.6-8.2)
Group 32.8 (95% CI 1.9-4.4)
Group 42.1 (95% CI 1.7-2.7)

Observational data: associations, not effects.

Further findings

Independent predictors of death (multivariable analysis)

Age: HR of death
3.5 (1.8-7.0) (95% CI)
Resection status: HR of death
5.5 (2.7-11.1) (95% CI)
Tumor stage: HR of death
1.9 (1.2-3.0) (95% CI)
Ki67 index: HR of death
1.7 (1.2-2.4) (95% CI)

Infants <4 years

More often low-risk constellations with better overall survival (OS) for all tumor stages

Original publication

Assessment of prognostic factors in pediatric adrenocortical tumors: the modified pediatric S-GRAS score in an international multicenter cohort—a work from the ENSAT-PACT working group. Eur J Endocrinol. Published 25 June 2024. doi:10.1093/ejendo/lvae079

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