{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,14]],"date-time":"2026-09-14T20:18:36Z","timestamp":1789417116232,"version":"build-2803163510"},"reference-count":37,"publisher":"Oxford University Press (OUP)","issue":"1","license":[{"start":{"date-parts":[[2024,6,25]],"date-time":"2024-06-25T00:00:00Z","timestamp":1719273600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"funder":[{"name":"IZKF","award":["Z-02CSP\/23"],"award-info":[{"award-number":["Z-02CSP\/23"]}]},{"DOI":"10.13039\/501100005973","name":"Dr. Mildred Scheel Stiftung f\u00fcr Krebsforschung","doi-asserted-by":"publisher","award":["70113303-6"],"award-info":[{"award-number":["70113303-6"]}],"id":[{"id":"10.13039\/501100005973","id-type":"DOI","asserted-by":"publisher"}]},{"name":"DFG German Research Foundation","award":["314061271-TRR 205"],"award-info":[{"award-number":["314061271-TRR 205"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2024,7,2]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Objective<\/jats:title>\n                  <jats:p>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.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Design<\/jats:title>\n                  <jats:p>Analysis of pS-GRAS items of pACC from 6 countries in collaboration of ENSAT-PACT, GPOH-MET, and IC-PACT.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods<\/jats:title>\n                  <jats:p>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; \u226520% = 2 points), resection status (R0 = 0; RX\/R1\/R2 = 1 point), age (&amp;lt;4 years = 0; \u22654 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).<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>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 &amp;lt; 4 years showed more often low-risk constellations with a better OS for all tumor stages.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusion<\/jats:title>\n                  <jats:p>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.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/ejendo\/lvae079","type":"journal-article","created":{"date-parts":[[2024,6,26]],"date-time":"2024-06-26T17:41:43Z","timestamp":1719423703000},"page":"64-74","source":"Crossref","is-referenced-by-count":14,"title":["Assessment of prognostic factors in pediatric adrenocortical tumors: the modified pediatric S-GRAS score in an international multicenter cohort\u2014a work from the ENSAT-PACT working group"],"prefix":"10.1093","volume":"191","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3273-1794","authenticated-orcid":false,"given":"Maria","family":"Riedmeier","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Division of Pediatric Hematology, Oncology and Stem Cell Transplantation, University Hospital W\u00fcrzburg, University of Wuerzburg , Josef-Schneiderstr. 2, 97080 Wuerzburg , Germany"},{"name":"KIONET, The Phase 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of Pediatric Oncology, Hacettepe University Faculty of Medicine , 06100 Ankara , Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6170-6398","authenticated-orcid":false,"given":"Martin","family":"Fassnacht","sequence":"additional","affiliation":[{"name":"Comprehensive Cancer Centre Mainfranken, University of Wuerzburg Medical Centre , Josef-Schneiderstr. 2, 97080 Wuerzburg , Germany"},{"name":"Department of Medicine, Division of Endocrinology and Diabetes, University of Wuerzbrug Medical Centre , Josef-Schneiderstr. 2, 97080 Wuerzburg , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bonald C","family":"Figueiredo","sequence":"additional","affiliation":[{"name":"Pel\u00e9 Pequeno Pr\u00edncipe Research Institute and Pequeno Pr\u00edncipe Faculty , Silva Jardim Avenue, \u00c1gua Verde, Curitiba, PR 80.250-200 , 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Wuerzburg , Josef-Schneiderstr. 2, 97080 Wuerzburg , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jan","family":"Idkowiak","sequence":"additional","affiliation":[{"name":"Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham , Birmingham B152TT , United Kingdom"},{"name":"Department of Endocrinology and Diabetes, Birmingham Women's and Children's NHS Foundation Trust , Birmingham, Birmingham B46NH , United Kingdom"},{"name":"Centre of Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, University of Birmingham , Birmingham B15 2TT , United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4577-0503","authenticated-orcid":false,"given":"Michaela","family":"Kuhlen","sequence":"additional","affiliation":[{"name":"KIONET, The Phase I\/II Pediatric Oncology Network Bavaria , 91054 Erlangen , Germany"},{"name":"Pediatrics and Adolescents 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