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

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.