Diagnostic work-up in paediatric adrenocortical tumours: an international consensus
Antonini, Sonir R Wiegering, Verena Halah, Mariana P Pinto, Luís Eduardo Cruvinel Attapatu, Navoda Kubat, Katja Dumic Elias, Jorge Eker, Nursah Fassnacht, Martin Fragoso, Maria C B V
Antonini, Sonir R
Wiegering, Verena
Halah, Mariana P
Pinto, Luís Eduardo Cruvinel
Attapatu, Navoda
Kubat, Katja Dumic
Elias, Jorge
Eker, Nursah
Fassnacht, Martin
Fragoso, Maria C B V
Abstract
OBJECTIVE: Paediatric adrenocortical tumours (pACTs) are rare but potentially life-threatening neoplasms, most of which are hormonally active and frequently present with androgen excess. Although a comprehensive evaluation is recommended, no consensus exists on the optimal diagnostic work-up. The goal of this document is to provide evidence-based recommendations to standardize the diagnostic evaluation of children with suspected pACTs.
METHODS: We applied a modified three-step Delphi process with an international panel of pACT experts to develop consensus on the initial diagnostic work-up. Following a literature review, the steering committee formulated statements, which were distributed electronically to 28 experts from Europe, the Americas, and Asia. Agreement was rated on a six-point Likert scale; ≥70% agreement was defined as consensus. Between rounds, results and feedback were reviewed within the ENS@T-KIDS group. In round one, 33 statements achieved consensus; 12 were revised and re-voted in round 2, with 7 reaching consensus. Five statements were further modified and one reached consensus in round three.
RESULTS: Response rates were 86% (n = 24) in round 1, 82% (n = 23) in round 2, and 86% (n = 24) in round 3. Forty-one statements received consensus covering 4 domains: (I) General Aspects and Clinical Assessment (17), (II) Endocrine Work-up (15), (III) Imaging (4), and (IV) Genetics (6).
CONCLUSION: The diagnostic evaluation of pACTs varies widely between centres and countries, influenced by resources and local expertise. This work delivers the first expert-derived, international consensus statements outlining a practical and standardized diagnostic pathway to support timely and consistent evaluation of children with suspected pACTs.
MIDER Authors
Affiliations
Date
2026-07-01
Type
Article
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Citation
Antonini SR, Wiegering V, Halah MP, Pinto LEC, Attapatu N, Kubat KD, Elias J Jr, Eker N, Fassnacht M, Fragoso MCBV, Fukami M, Guercio G, Gönç EN, Guran T, Huebner A, Janus D, Krone N, Kuhlen M, Lam CG, Muglia VF, Meena JP, Ozon ZA, Pereira RM, Del Rivero J, Schloetelburg W, Tuli G, Waguespack SG, Wudy SA, Yalcin B, Idkowiak J. Diagnostic work-up in paediatric adrenocortical tumours: an international consensus. Eur J Endocrinol. 2026 Jul 1;195(1):R1-R22. doi: 10.1093/ejendo/lvag086.