Diagnostic work-up in paediatric adrenocortical tumours: an international consensus.

Authors

Sonir R Antonini, Verena Wiegering, Mariana P Halah, Luís Eduardo Cruvinel Pinto, Navoda Attapatu, Katja Dumic Kubat, Jorge Elias, Nursah Eker, Martin Fassnacht, Maria C B V Fragoso, Maki Fukami, Gabriela Guercio, Elmas Nazlı Gönç, Tulay Guran, Angela Huebner, Dominika Janus, Nils Krone, Michaela Kuhlen, Catherine G Lam, Valdair F Muglia, Jagdish P Meena, Zeynep Alev Ozon, Rosana Marques Pereira, Jaydira Del Rivero, Wiebke Schloetelburg, Gerdi Tuli, Steven G Waguespack, Stefan A Wudy, Bilgehan Yalcin, Jan Idkowiak

Published in

European journal of endocrinology. Volume 195. Issue 1. Pages R1-R22. Jul 01, 2026.

Abstract

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.
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.
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).
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.

PMID:
42413072
Bibliographic data and abstract were imported from PubMed on 08 Jul 2026.

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