Open Access

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

• 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 roundPaediatric 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.…