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-Reference-Cited by-同舟云学术

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

Published:2024-06-25 Issue:1 Volume:191 Page:64-74
ISSN:0804-4643
Container-title:European Journal of Endocrinology
language:en
Short-container-title:

Author:

Riedmeier Maria¹²^ORCID,Agarwal Shipra³,Antonini Sonir⁴,Costa Tatiana E I Jaick B⁵,Diclehan Orhan⁶,Fassnacht Martin⁷⁸^ORCID,Figueiredo Bonald C⁹,Guran Tulay¹⁰^ORCID,Härtel Christoph¹,Haubitz Imme¹,Idkowiak Jan¹¹¹²¹³,Kuhlen Michaela²¹⁴^ORCID,Noronha Lúcia¹⁵,Parise Ivy Zortéa S⁵,Redlich Antje²¹⁶^ORCID,Puglisi Soraya¹⁷^ORCID,Saniye Ekinci⁶,Schlegel Paul-Gerhardt¹²⁷,Yalcin Bilgehan⁶,Wiegering Verena¹²⁷¹⁸

Affiliation:

1. Department of Pediatrics, Division of Pediatric Hematology, Oncology and Stem Cell Transplantation, University Hospital Würzburg, University of Wuerzburg , Josef-Schneiderstr. 2, 97080 Wuerzburg , Germany

2. KIONET, The Phase I/II Pediatric Oncology Network Bavaria , 91054 Erlangen , Germany

3. Department of Pathology, All India Institute of Medical Sciences , New Delhi 110029 , India

4. Department of Pediatrics, Ribeirao Preto Medical School, University of Sao Paulo , Ribeirao Preto, Sao Paulo 14051-200 , Brazill

5. Hospital Infantil Joana Gusmão , Department of Pediatrics, 152 Rui Barbosa St., Florianópolis, SC 88025-300 , Brazil

6. Department of Pediatric Oncology, Hacettepe University Faculty of Medicine , 06100 Ankara , Turkey

7. Comprehensive Cancer Centre Mainfranken, University of Wuerzburg Medical Centre , Josef-Schneiderstr. 2, 97080 Wuerzburg , Germany

8. Department of Medicine, Division of Endocrinology and Diabetes, University of Wuerzbrug Medical Centre , Josef-Schneiderstr. 2, 97080 Wuerzburg , Germany

9. Pelé Pequeno Príncipe Research Institute and Pequeno Príncipe Faculty , Silva Jardim Avenue, Água Verde, Curitiba, PR 80.250-200 , Brazil

10. Department of Pediatric Endocrinology and Diabetes Istanbul, Marmara University School of Medicine , Istanbul 34722 , Turkey

11. Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham , Birmingham B152TT , United Kingdom

12. Department of Endocrinology and Diabetes, Birmingham Women's and Children's NHS Foundation Trust , Birmingham, Birmingham B46NH , United Kingdom

13. Centre of Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, University of Birmingham , Birmingham B15 2TT , United Kingdom

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

15. Serviço de Anatomia Patológica, Hospital de Clínicas, Universidade Federal do Paraná , 181 General Carneiro, Alto da Glória, Curitiba, PR 80060-900 , Brazil

16. Pediatric Oncology, Otto-von-Guericke-University , 39120 Magdeburg , Germany

17. Internal Medicine, Department of Clinical and Biological Sciences, S. Luigi Gonzaga Hospital, University of Turin , Orbassano 10043 , Italy

18. Mildred Scheel Early Career Center, University Hospital Wuerzburg , 97080 Wuerzburg , Germany

Abstract

Abstract Objective Pediatric adrenocortical carcinoma (pACC) is rare, and prognostic stratification remains challenging. We aimed to confirm the prognostic value of the previously published pediatric scoring system (pS-GRAS) in an international multicenter cohort. Design Analysis of pS-GRAS items of pACC from 6 countries in collaboration of ENSAT-PACT, GPOH-MET, and IC-PACT. Methods We received patient data of the pS-GRAS items including survival information from 9 centers. PS-GRAS score was calculated as a sum of tumor stage (1 = 0; 2-3 = 1; 4 = 2 points), grade (Ki67 index: 0%-9% = 0; 10%-19% = 1; ≥20% = 2 points), resection status (R0 = 0; RX/R1/R2 = 1 point), age (<4 years = 0; ≥4 years = 1 point), and hormone production (androgen production = 0; glucocorticoid-/mixed-/no-hormone production = 1 point) generating 8 scores and 4 groups (1: 0-2, 2: 3-4, 3: 5, 4: 6-7). Primary endpoint was overall survival (OS). Results We included 268 patients with median age of 4 years. The analysis of the pS-GRAS score showed a significantly favorable prognosis in patients with a lower scoring compared to higher scoring groups (5-year OS: Group 1 98%; group 2 87% [hazard ratio {HR} of death 3.6, 95% CI of HR 1.6-8.2]; group 3 43% [HR of death 2.8, 95% CI 1.9-4.4]; group 4: OS 18% [HR of death 2.1, 95% CI 1.7-2.7]). In the multivariable analysis, age (HR of death 3.5, 95% CI 1.8-7.0), resection status (HR of death 5.5, 95% CI 2.7-11.1), tumor stage (HR of death 1.9, 95% CI of HR 1.2-3.0), and Ki67 index (HR of death 1.7, 95% CI 1.2-2.4) remained strong independent outcome predictors. Especially infants < 4 years showed more often low-risk constellations with a better OS for all tumor stages. 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.

Funder

IZKF

Dr. Mildred Scheel Stiftung für Krebsforschung

DFG German Research Foundation

Publisher

Oxford University Press (OUP)

Link

https://academic.oup.com/ejendo/advance-article-pdf/doi/10.1093/ejendo/lvae079/58341760/lvae079.pdf

Reference37 articles.

1. Increase in childhood carcinomas in north-west England;Birch;Lancet,1988

2. Cancer incidence among children in France, 1990-1999;Desandes;Pediatr Blood Cancer,2004

3. An inherited p53 mutation that contributes in a tissue-specific manner to pediatric adrenal cortical carcinoma;Ribeiro;Proc Natl Acad Sci U S A,2001

4. Impact of neonatal screening and surveillance for the TP53 R337H mutation on early detection of childhood adrenocortical tumors;Custódio;J Clin Oncol,2013