Authors
Fragoso Maria, Van de Ven Kees, Riedmeier Maria, Abib Simone, Agarwal Shipra, Antoniazzi Augusto Perazollo, Antonini Sonir, G Suren Arul, Baecklund Fredrik, Brandalise Silvia, Daiggi Camila Maria Martin, Deal Cheri, Del Rivero Jaydira, Denes Francisco Tibor, Dumic Katja, Ekinci Saniye, El Jaick Tatiana, Engstler Gernot, Fassnacht Martin, Figueiredo Bonald Cavalcante, Flores Paula, Fortunati Daniela, Galuzzo Laura, Gönç Nazlı, Górecki Wojciech, Guercio Gabriela, Gültekin Melis, Guran Tulay, Haak Harm, Hendriks Emilie, Idkowiak Jan, Janus Dominika, Kremer Vilani, Krueger Johanna, G Lam Catherine, Luiz Fernando Lopes, Malkin David, Manousaki Despoina, Mattone Maria Celeste, Marks Stephen, Molina Carlos, Munarin Jessica, Ohmoto Akihiro, Pamporaki Christina, Pianovski Mara, Piché Nelson, Perier Nancy, Ribeiro Raul, Ribeiro Rodrigo Chaves, Rodrigues Karla, Taczanowska-Niemczuk Anna, D R Thompson Lester, Tuli Gerdi, Van Noesel Max, Virgone Calogero, G Waguespack Steven, Walz Martin, Wasserman Jonathan, Wiegering Armin, Yalcin Bilgehan, R de Krijger Ronald, Wiegering Verena
Published in
European journal of endocrinology. Jun 22, 2026. Epub Jun 22, 2026.
Abstract
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 three-step Delphi consensus method was used to finalize the statements from an international panel of pediatric ACC experts from four 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 six-point Likert scale with the opportunity to provide structured feedback. Consensus was defined a priori as ≥75% 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.
PMID:
42324800
Bibliographic data and abstract were imported from PubMed on 22 Jun 2026.
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