{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,9]],"date-time":"2026-07-09T10:19:55Z","timestamp":1783592395686,"version":"3.55.0"},"reference-count":25,"publisher":"Bioscientifica","issue":"5","funder":[{"DOI":"10.13039\/501100009379","name":"Interdisziplin\u00e4res Zentrum f\u00fcr Klinische Forschung, Universit\u00e4tsklinikum W\u00fcrzburg","doi-asserted-by":"publisher","award":["Z-02CSP\/23"],"award-info":[{"award-number":["Z-02CSP\/23"]}],"id":[{"id":"10.13039\/501100009379","id-type":"DOI","asserted-by":"publisher"}]},{"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"}]},{"DOI":"10.13039\/501100001659","name":"Deutsche Forschungsgemeinschaft","doi-asserted-by":"publisher","award":["314061271-TRR 205"],"award-info":[{"award-number":["314061271-TRR 205"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["journals.bioscientifica.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2025,5,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Therapeutic options of advanced pediatric adrenocortical carcinoma (pACC) are limited, and achieving valuable risk stratification remains challenging. We refined the value of prognostic factors with an emphasis on resection status. Retrospective international data from 106 patients with advanced pACC from various collaborating centers of the international pACC working groups ENSAT-PACT, IC-PACT and\/or from individual international collaboration diagnosed were collected. One hundred six patients aged 0.1\u201318.1 (median 7.6) years were diagnosed with pACC, with 42 tumor stage III and 64 stage IV, respectively. Eighty percent (85\/106) of the tumors were hormone-producing, with a mean Ki67 index for both stage groups of 29%. Patient survival was 45% (48\/106) with a mean follow-up of 17.7 months. Higher age, tumor stage IV and increased Ki67 index worsened the prognosis on overall survival. Resection status had an essential impact on survival, as the patients with R0 resection (n = 32) had a better overall survival (71% for stage III patients; 80% for stage IV patients) than patients with R1 (n = 24) (45% for stage III; 69% for stage IV), R2 (n = 33) (17% for stage III; 15% for stage IV) and Rx (n = 7) (0% for stage III; 17% for stage IV). Of the ten patients with tumor spillage, only a few (57% of stage III; 0% of stage IV patients) survived. The resection status has a significant impact on overall survival in pACC. Therefore, tumor surgery should only be undertaken by experienced surgeons proficient in adrenalectomy and oncology, ideally within specialized pediatric oncological centers with a multidisciplinary team setting.<\/jats:p>","DOI":"10.1530\/erc-24-0135","type":"journal-article","created":{"date-parts":[[2025,3,24]],"date-time":"2025-03-24T09:49:06Z","timestamp":1742809746000},"update-policy":"https:\/\/doi.org\/10.1530\/crossmarkpolicy-8","source":"Crossref","is-referenced-by-count":1,"title":["Evaluation of prognostic factors in advanced pediatric ACC"],"prefix":"10.1530","volume":"32","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3273-1794","authenticated-orcid":true,"given":"Maria","family":"Riedmeier","sequence":"first","affiliation":[{"name":"University Hospital W\u00fcrzburg, Department of Pediatrics, Division of Pediatric Hematology, Oncology and Stem Cell Transplantation, University of Wuerzburg 1 , ,","place":["Wuerzburg, Germany"]},{"name":"KIONET, The Phase I\/II Pediatric Oncology Network Bavaria, W\u00fcrzburg, Erlangen, Regensburg, Augsburg, M\u00fcnchen, Germany 2"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shipra","family":"Agarwal","sequence":"additional","affiliation":[{"name":"Department of Pathology, All India Institute of Medical Sciences 3 , ,","place":["New Delhi, India"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4778-8803","authenticated-orcid":true,"given":"Sonir R R","family":"Antonini","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Ribeirao Preto Medical School, University of Sao Paulo 4 , ,","place":["Sao Paulo, Brazil"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Saniye","family":"Ekinci","sequence":"additional","affiliation":[{"name":"Pediatric Surgery, Department of Pediatric Surgery, Hacettepe University Faculty of Medicine 5 , ,","place":["Ankara, Turkey"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martin","family":"Fassnacht","sequence":"additional","affiliation":[{"name":"Comprehensive Cancer Centre Mainfranken, University of Wuerzburg Medical Centre 6 , ,","place":["Wuerzburg, Germany"]},{"name":"Department of Medicine, Division of Endocrinology and Diabetes, University of Wuerzbrug Medical Centre 7 , ,","place":["Wuerzburg, Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bonald Cavalcante","family":"Figueiredo","sequence":"additional","affiliation":[{"name":"Pel\u00e9 Pequeno Pr\u00edncipe Research Institute and Pequeno Pr\u00edncipe Faculty 8 , ,","place":["Curitiba, Brazil"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christoph","family":"H\u00e4rtel","sequence":"additional","affiliation":[{"name":"University Hospital W\u00fcrzburg, Department of Pediatrics, Division of Pediatric Hematology, Oncology and Stem Cell Transplantation, University of Wuerzburg 1 , ,","place":["Wuerzburg, Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jagdish Prasad","family":"Meena","sequence":"additional","affiliation":[{"name":"Division of Pediatric Oncology, Department of Pediatrics, Mother & Child Block, All India Institute of Medical Sciences 9 , ,","place":["New Delhi, India"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephen D","family":"Marks","sequence":"additional","affiliation":[{"name":"Department of Paediatric Nephrology, Great Ormond Street Hospital for Children NHS Foundation Trust 10 , ,","place":["London, UK"]},{"name":"NIHR Great Ormond Street Hospital Biomedical Research Centre, University College London Great Ormond Street Institute of Child Health 11 , ,","place":["London, UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jessica","family":"Munarin","sequence":"additional","affiliation":[{"name":"Department of Pediatric Endocrinology, Regina Margherita Children\u2019s Hospital 12 , ,","place":["Turin, Italy"]},{"name":"Department of Pediatrics, University of Turin 13 , ,","place":["Turin, Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Soraya","family":"Puglisi","sequence":"additional","affiliation":[{"name":"Internal Medicine, Department of Clinical and Biological Sciences, S Luigi Gonzaga Hospital, University of Turin 14 , ,","place":["Orbassano, Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gerdi","family":"Tuli","sequence":"additional","affiliation":[{"name":"Department of Pediatric Endocrinology, Regina Margherita Children\u2019s Hospital 12 , ,","place":["Turin, Italy"]},{"name":"Department of Pediatrics, University of Turin 13 , ,","place":["Turin, Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bilgehan","family":"Yalcin","sequence":"additional","affiliation":[{"name":"Department of Pediatric Oncology, Hacettepe University Faculty of Medicine 15 , ,","place":["Ankara, Turkey"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul G","family":"Schlegel","sequence":"additional","affiliation":[{"name":"University Hospital W\u00fcrzburg, Department of Pediatrics, Division of Pediatric Hematology, Oncology and Stem Cell Transplantation, University of Wuerzburg 1 , ,","place":["Wuerzburg, Germany"]},{"name":"KIONET, The Phase I\/II Pediatric Oncology Network Bavaria, W\u00fcrzburg, Erlangen, Regensburg, Augsburg, M\u00fcnchen, Germany 2"},{"name":"Pediatric Surgery, Department of Pediatric Surgery, Hacettepe University Faculty of Medicine 5 , ,","place":["Ankara, Turkey"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Armin","family":"Wiegering","sequence":"additional","affiliation":[{"name":"Comprehensive Cancer Centre Mainfranken, University of Wuerzburg Medical Centre 6 , ,","place":["Wuerzburg, Germany"]},{"name":"Department of General, Visceral, Transplant, Vascular and Pediatric Surgery, W\u00fcrzburg University Hospital 16 , ,","place":["W\u00fcrzburg, Germany"]},{"name":"Department of Biochemistry and Molecular Biology, University of W\u00fcrzburg 17 , ,","place":["W\u00fcrzburg, Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2802-4034","authenticated-orcid":true,"given":"Verena","family":"Wiegering","sequence":"additional","affiliation":[{"name":"University Hospital W\u00fcrzburg, Department of Pediatrics, Division of Pediatric Hematology, Oncology and Stem Cell Transplantation, University of Wuerzburg 1 , ,","place":["Wuerzburg, Germany"]},{"name":"KIONET, The Phase I\/II Pediatric Oncology Network Bavaria, W\u00fcrzburg, Erlangen, Regensburg, Augsburg, M\u00fcnchen, Germany 2"},{"name":"Pediatric Surgery, Department of Pediatric Surgery, Hacettepe University Faculty of Medicine 5 , ,","place":["Ankara, Turkey"]},{"name":"Mildred Scheel Early Career Center, University Hospital Wuerzburg 18 , ,","place":["Wuerzburg, Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"416","published-online":{"date-parts":[[2025,3,24]]},"reference":[{"key":"2026070905415138700_bib1","doi-asserted-by":"crossref","first-page":"6641","DOI":"10.3390\/jcm11226641","article-title":"Retrospective analysis of prognostic factors in pediatric patients with adrenocortical tumor from unique tertiary center with long-term follow-up","volume":"11","author":"Bachega","year":"2022","journal-title":"J Clin Med"},{"key":"2026070905415138700_bib2","doi-asserted-by":"crossref","first-page":"2345","DOI":"10.1200\/JCO.2014.59.5728","article-title":"Revisiting Li-fraumeni syndrome from TP53 mutation carriers","volume":"33","author":"Bougeard","year":"2015","journal-title":"J Clin Oncol"},{"key":"2026070905415138700_bib3","doi-asserted-by":"crossref","first-page":"284","DOI":"10.2350\/08-06-0489.1","article-title":"Adrenal cortical neoplasms in children: why so many carcinomas and yet so many survivors?","volume":"12","author":"Dehner","year":"2009","journal-title":"Pediatr Dev Pathol"},{"key":"2026070905415138700_bib4","doi-asserted-by":"crossref","first-page":"G1","DOI":"10.1530\/EJE-18-0608","article-title":"European society of endocrinology clinical practice guidelines on the management of adrenocortical carcinoma in adults, in collaboration with the European network for the study of adrenal tumors","volume":"179","author":"Fassnacht","year":"2018","journal-title":"Eur J Endocrinol"},{"key":"2026070905415138700_bib5","doi-asserted-by":"crossref","first-page":"143","DOI":"10.1055\/s-0032-1304627","article-title":"Surgical aspects in the treatment of adrenocortical carcinomas in children: data of the GPOH-MET 97 trial","volume":"224","author":"Hubertus","year":"2012","journal-title":"Klin Padiatr"},{"key":"2026070905415138700_bib6","doi-asserted-by":"crossref","first-page":"2836","DOI":"10.3892\/or.2014.3506","article-title":"Adrenocortical carcinoma in children: first population-based clinicopathological study with long-term follow-up","volume":"32","author":"Kerkhofs","year":"2014","journal-title":"Oncol Rep"},{"key":"2026070905415138700_bib7","doi-asserted-by":"crossref","first-page":"150","DOI":"10.1016\/j.surg.2017.05.024","article-title":"Fifteen years of adrenalectomies: impact of specialty training and operative volume","volume":"163","author":"Lindeman","year":"2018","journal-title":"Surgery"},{"key":"2026070905415138700_bib8","doi-asserted-by":"crossref","first-page":"288","DOI":"10.1007\/s12022-020-09654-x","article-title":"Clinical impact of pathological features including the Ki-67 labeling index on diagnosis and prognosis of adult and pediatric adrenocortical tumors","volume":"32","author":"Martins-Filho","year":"2021","journal-title":"Endocr Pathol"},{"key":"2026070905415138700_bib9","doi-asserted-by":"crossref","first-page":"1025","DOI":"10.1016\/j.jpedsurg.2013.02.017","article-title":"Predictors of survival in pediatric adrenocortical carcinoma: a surveillance, epidemiology, and end results (SEER) program study","volume":"48","author":"Mcateer","year":"2013","journal-title":"J Pediatr Surg"},{"key":"2026070905415138700_bib10","doi-asserted-by":"crossref","first-page":"2004","DOI":"10.1016\/S0022-5347(01)66673-4","article-title":"Clinical, hormonal and pathological findings in a comparative study of adrenocortical neoplasms in childhood and adulthood","volume":"154","author":"Mendonca","year":"1995","journal-title":"J Urol"},{"key":"2026070905415138700_bib11","doi-asserted-by":"crossref","first-page":"838","DOI":"10.1200\/JCO.2004.08.085","article-title":"Clinical and outcome characteristics of children with adrenocortical tumors: a report from the International Pediatric Adrenocortical Tumor Registry","volume":"22","author":"Michalkiewicz","year":"2004","journal-title":"J Clin Oncol"},{"key":"2026070905415138700_bib12","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1111\/cen.13021","article-title":"Adrenal surgery in England: better outcomes in high-volume practices","volume":"85","author":"Palazzo","year":"2016","journal-title":"Clin Endocrinol"},{"key":"2026070905415138700_bib13","doi-asserted-by":"crossref","first-page":"1060","DOI":"10.1001\/archsurg.2009.191","article-title":"Outcomes from 3144 adrenalectomies in the United States: which matters more, surgeon volume or specialty?","volume":"144","author":"Park","year":"2009","journal-title":"Arch Surg"},{"key":"2026070905415138700_bib14","doi-asserted-by":"crossref","first-page":"647","DOI":"10.1590\/S0004-27302004000500009","article-title":"Founder effect for the highly prevalent R337H mutation of tumor suppressor p53 in Brazilian patients with adrenocortical tumors","volume":"48","author":"Pinto","year":"2004","journal-title":"Arq Bras Endocrinol Metabol"},{"key":"2026070905415138700_bib15","doi-asserted-by":"crossref","first-page":"366","DOI":"10.1055\/s-0032-1327579","article-title":"Systemic treatment of adrenocortical carcinoma in children: data from the German GPOH-MET 97 trial","volume":"224","author":"Redlich","year":"2012","journal-title":"Klin Padiatr"},{"key":"2026070905415138700_bib16","doi-asserted-by":"crossref","first-page":"363","DOI":"10.1097\/SLA.0b013e3182367ac3","article-title":"Impact of lymphadenectomy on the oncologic outcome of patients with adrenocortical carcinoma","volume":"255","author":"Reibetanz","year":"2012","journal-title":"Ann Surg"},{"key":"2026070905415138700_bib17","doi-asserted-by":"crossref","first-page":"9330","DOI":"10.1073\/pnas.161479898","article-title":"An inherited p53 mutation that contributes in a tissue-specific manner to pediatric adrenal cortical carcinoma","volume":"98","author":"Ribeiro","year":"2001","journal-title":"Proc Natl Acad Sci U S A"},{"key":"2026070905415138700_bib18","doi-asserted-by":"crossref","first-page":"5266","DOI":"10.3390\/cancers13215266","article-title":"Adrenocortical carcinoma in childhood: a systematic review","volume":"13","author":"Riedmeier","year":"2021","journal-title":"Cancers"},{"key":"2026070905415138700_bib19","doi-asserted-by":"crossref","first-page":"751","DOI":"10.1530\/EJE-22-0173","article-title":"Assessment of prognostic factors in pediatric adrenocortical tumors: a systematic review and evaluation of a modified S-GRAS score","volume":"187","author":"Riedmeier","year":"2022","journal-title":"Eur J Endocrinol"},{"key":"2026070905415138700_bib20","doi-asserted-by":"crossref","first-page":"2463","DOI":"10.1200\/JCO.20.02871","article-title":"Treatment of pediatric adrenocortical carcinoma with surgery, retroperitoneal lymph node dissection, and chemotherapy: the children's oncology group ARAR0332 protocol","volume":"39","author":"Rodriguez-Galindo","year":"2021","journal-title":"J Clin Oncol"},{"key":"2026070905415138700_bib21","doi-asserted-by":"crossref","first-page":"e945","DOI":"10.1542\/peds.2013-3926","article-title":"Cancer incidence rates and trends among children and adolescents in the United States, 2001\u20132009","volume":"134","author":"Siegel","year":"2014","journal-title":"Pediatrics"},{"key":"2026070905415138700_bib22","doi-asserted-by":"crossref","DOI":"10.3389\/fendo.2022.914449","article-title":"Adrenalectomies in children and adolescents in Germany \u2013 a diagnose related groups based analysis from 2009-2017","volume":"13","author":"Uttinger","year":"2022","journal-title":"Front Endocrinol"},{"key":"2026070905415138700_bib23","doi-asserted-by":"crossref","first-page":"4619","DOI":"10.1200\/JCO.2008.17.2775","article-title":"Management of endocrine manifestations and the use of mitotane as a chemotherapeutic agent for adrenocortical carcinoma","volume":"27","author":"Veytsman","year":"2009","journal-title":"J Clin Oncol"},{"key":"2026070905415138700_bib24","doi-asserted-by":"crossref","first-page":"602","DOI":"10.1200\/JCO.2013.52.6863","article-title":"Prevalence and functional consequence of TP53 mutations in pediatric adrenocortical carcinoma: a children's oncology group study","volume":"33","author":"Wasserman","year":"2015","journal-title":"J Clin Oncol"},{"key":"2026070905415138700_bib25","doi-asserted-by":"crossref","DOI":"10.1002\/pbc.28836","article-title":"Clinical prognostic factors in pediatric adrenocortical tumors: a meta-analysis","volume":"68","author":"Zambaiti","year":"2021","journal-title":"Pediatr Blood Cancer"}],"container-title":["Endocrine-Related Cancer"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/erc.bioscientifica.com\/view\/journals\/erc\/32\/5\/ERC-24-0135.xml","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/erc.bioscientifica.com\/downloadpdf\/journals\/erc\/32\/5\/ERC-24-0135.xml","content-type":"text\/html","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/journals.bioscientifica.com\/erc\/article-pdf\/doi\/10.1530\/ERC-24-0135\/144830\/erc-24-0135.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/journals.bioscientifica.com\/erc\/article-pdf\/doi\/10.1530\/ERC-24-0135\/144830\/erc-24-0135.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,7,9]],"date-time":"2026-07-09T09:42:04Z","timestamp":1783590124000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.bioscientifica.com\/erc\/article\/32\/5\/e240135\/25889\/Evaluation-of-prognostic-factors-in-advanced"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,3,24]]},"references-count":25,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2025,5,1]]}},"URL":"https:\/\/doi.org\/10.1530\/erc-24-0135","relation":{},"ISSN":["1479-6821","1351-0088"],"issn-type":[{"value":"1479-6821","type":"electronic"},{"value":"1351-0088","type":"print"}],"subject":[],"published":{"date-parts":[[2025,3,24]]},"article-number":"e240135"}}