International Delphi-based consensus: therapeutic approaches for pediatric adrenocortical carcinoma
Maria, Fragoso Kees, Van de Ven Maria, Riedmeier Simone, Abib Shipra, Agarwal Perazollo, Antoniazzi Augusto Sonir, Antonini Suren Arul, G Fredrik, Baecklund Silvia, Brandalise
Maria, Fragoso
Kees, Van de Ven
Maria, Riedmeier
Simone, Abib
Shipra, Agarwal
Perazollo, Antoniazzi Augusto
Sonir, Antonini
Suren Arul, G
Fredrik, Baecklund
Silvia, Brandalise
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.
MIDER Authors
Affiliations
Date
2026-06-22
Type
Article
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Citation
Maria F, Kees VV, Maria R, Simone A, Shipra A, Perazollo AA, Sonir A, Suren Arul G, Fredrik B, Silvia B, Martin DCM, Cheri D, Jaydira DR, Tibor DF, Katja D, Saniye E, Tatiana EJ, Gernot E, Martin F, Cavalcante FB, Paula F, Daniela F, Laura G, Nazlı G, Wojciech G, Gabriela G, Melis G, Tulay G, Harm H, Emilie H, Jan I, Dominika J, Vilani K, Johanna K, Lam Catherine G, Lopes LF, David M, Despoina M, Celeste MM, Stephen M, Carlos M, Jessica M, Akihiro O, Christina P, Mara P, Nelson P, Nancy P, Raul R, Chaves RR, Karla R, Anna TN, Thompson Lester DR, Gerdi T, Max VN, Calogero V, Waguespack Steven G, Martin W, Jonathan W, Armin W, Bilgehan Y, de Krijger Ronald R, Verena W. International Delphi-based Consensus: Therapeutic Approaches for Pediatric Adrenocortical Carcinoma. Eur J Endocrinol. 2026 Jun 22:lvag101. doi: 10.1093/ejendo/lvag101. Epub ahead of print