{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,27]],"date-time":"2026-07-27T14:17:33Z","timestamp":1785161853190,"version":"3.55.0"},"reference-count":167,"publisher":"Oxford University Press (OUP)","issue":"1","license":[{"start":{"date-parts":[[2026,6,22]],"date-time":"2026-06-22T00:00:00Z","timestamp":1782086400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"funder":[{"name":"Interdisziplin\u00e4res Zentrum f\u00fcr klinische Forschung","award":["Z-02SCP\/23"],"award-info":[{"award-number":["Z-02SCP\/23"]}]},{"name":"Mildred Scheel program","award":["70113303-6"],"award-info":[{"award-number":["70113303-6"]}]},{"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":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,7,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Adrenocortical carcinoma (ACC) in children is a rare and aggressive malignancy. Despite representing a small fraction of pediatric adrenal tumors, ACC poses distinct challenges due to its heterogeneous biological behavior, frequent hormone secretion, and the limited availability of standardized, pediatric-specific treatment protocols. Complete surgical resection remains the cornerstone of curative intent therapy, and complete adrenalectomy offers the best chance for long-term survival. Given the aggressiveness of ACC, particularly in advanced disease, adjuvant therapies are often considered to improve outcomes. The aim of this consensus meeting was to bring together international experts to develop international guidance based on the best available evidence and collective clinical experience to harmonize long-term management. A modified 3-step Delphi consensus method was used to finalize the statements from an international panel of pediatric ACC experts from 4 continents (Europe, the Americas, and Asia, and Oceania). An electronic survey was developed by the steering committee (ENSAT Kids) and distributed to the panel. Agreement and disagreement were rated using a 6-point Likert scale with the opportunity to provide structured feedback. Consensus was defined a priori as \u226575% agreement. This Delphi process yielded consensus statements for therapy guidance, comprising 12 surgical statements and 68 medical treatment statements, organized by strategy and disease stage. These statements are intended to support clinicians in delivering more consistent and standardized therapeutic strategies for pediatric ACC. Future efforts should prioritize the design and conduct of high-quality, collaborative research studies in order to provide more evidence and therapy improvements.<\/jats:p>","DOI":"10.1093\/ejendo\/lvag101","type":"journal-article","created":{"date-parts":[[2026,6,16]],"date-time":"2026-06-16T12:39:23Z","timestamp":1781613563000},"page":"R23-R47","source":"Crossref","is-referenced-by-count":0,"title":["International Delphi-based consensus: therapeutic approaches for pediatric adrenocortical carcinoma"],"prefix":"10.1093","volume":"195","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6150-1915","authenticated-orcid":false,"given":"Fragoso","family":"Maria","sequence":"first","affiliation":[{"name":"Hospital das Cl\u00ednicas da Faculdade de Medicina, Universidade de S\u00e3o Paulo (HCFMUSP) , S\u00e3o Paulo, SP CEP 05403-000 ,","place":["Brasil"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Van","family":"de Ven Kees","sequence":"additional","affiliation":[{"name":"Pediatric Surgery, Princess Maxima Center for Pediatric Oncology , Postbus 113, 3720 AC Bilthoven, Heidelberglaan 25, Utrecht 3584 CS, The Nederlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3273-1794","authenticated-orcid":false,"given":"Riedmeier","family":"Maria","sequence":"additional","affiliation":[{"name":"University Hospital W\u00fcrzburg, Department of Pediatrics, Division of Pediatric Hematology, Oncology and Stem Cell Transplantation, University of Wuerzburg , Josef-Schneiderstr. 2, Wuerzburg 97080 ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Abib","family":"Simone","sequence":"additional","affiliation":[{"name":"Pediatric Oncology Institute, GRAACC, Federal University of S\u00e3o Paulo , Sao Paulo 04039-001 ,","place":["Brazil"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Agarwal","family":"Shipra","sequence":"additional","affiliation":[{"name":"Department of Pathology, AIIMS , New Delhi 10029 ,","place":["India"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antoniazzi Augusto","family":"Perazollo","sequence":"additional","affiliation":[{"name":"Department of Molecular Medicine and Surgery Stockholm, Karolinska Institute , Stockholm 171 77, Sweden"},{"name":"Karolinska University Hospital, Pediatric Oncology Stockholm , Stockholm 171 77, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4778-8803","authenticated-orcid":false,"given":"Antonini","family":"Sonir","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Pediatric Endocrinology Section Ribeirao Preto Medical School, University of Sao Paulo, Sao Paulo 05403-000 ,","place":["Brazil"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G","family":"Suren Arul","sequence":"additional","affiliation":[{"name":"Department of Paediatrics Surgeon, Birmingham Women and Children's Hospital , Birmingham B4 6NN, United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1333-2912","authenticated-orcid":false,"given":"Baecklund","family":"Fredrik","sequence":"additional","affiliation":[{"name":"Department of Molecular Medicine and Surgery, Karolinska Institutet , Stockholm 171 77 ,","place":["Sweden"]},{"name":"Pediatric Oncology, Karolinska University Hospital , Stockholm 171 77 ,","place":["Sweden"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brandalise","family":"Silvia","sequence":"additional","affiliation":[{"name":"Pediatric Department, The State University of Campinas (UNICAMP) , Sao Paulo 13.083-884, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daiggi Camila Maria","family":"Martin","sequence":"additional","affiliation":[{"name":"Domingos A Boldrini Children's Center for Hematologic Investigations, Oncology Campinas , Sao Paulo 13083-884 ,","place":["Brazil"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Deal","family":"Cheri","sequence":"additional","affiliation":[{"name":"Universit\u00e9 de Montr\u00e9al, Associate Member Research Center, CHU Ste-Justine , Montreal H3T 1C5, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9710-4030","authenticated-orcid":false,"given":"Del Rivero","family":"Jaydira","sequence":"additional","affiliation":[{"name":"Developmental Therapeutics Branch, National Cancer Institute, National Institutes of Health, Maryland, MD 20892-4564 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Denes Francisco","family":"Tibor","sequence":"additional","affiliation":[{"name":"Pediatric Urology Unit, Division of Urology, HCFMUSP - 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