ECEESPE2025 Poster Presentations Adrenal and Cardiovascular Endocrinology (169 abstracts)
Diagnostic work-up of paediatric adrenocortical tumours – International consensus through a modified Delphi process
Jan Idkowiak 1,2 , Verena Wiegering 3 , Navoda Attapatu 4 , Katja Dumic 5 , Nursah Eker 6 , Jorge Elias Jr 7 , Martin Fassnacht 8 , Maria Candida Barisson Villares Fragoso 9 , Maki Fukami 10 , E Nazlı Gönç 11 , Gabriela Guercio 12 , Tulay Guran 6 , Angela Hübner 13 , Dominka Janus 14 , Nils Krone 15 , Michaela Kuhlen 16 , Catherine Lam 17 , Jagdish Meena 18 , Valdair Muglia 9 , Alev Ozon 11 , Rosana Marques Pereira 19 , Jaydira Del Rivero 20 , Schlötelburg Wiebke 8 , Gerdi Tuli 21 , Steven Waguespack 22 , Stefan Wudy 23 , Bilgehan Yalcin 11 & Sonir R Antonini 7
1Birmingham Women’s and Children’s NHS FT, Endocrinology and Diabetes, Birmingham, UK; 2University of Birmingham, Metabolism and Systems Science, Birmingham, UK; 3Goethe University Frankfurt, Paediatric Haemtology and Oncology, Frankfurt, Germany; 4Lady Ridgeway Hospital, Colombo, Sri Lanka; 5University Clinical Center Zagreb, Department of Pediatric Endocrinology and Diabetes, Zagreb, Croatia; 6Marmara University School of Medicine, Istanbul, Türkiye; 7University of Sao Paulo, Sao Paulo, Brazil; 8University Hospital Würzburg, Department of Endocrinology, Wuerzburg, Germany; 9University of São Paulo, Sao Paulo, Brazil; 10National Research Institute for Child Health and Development, Tokyo, Japan; 11Hacettepe University, Ankara, Türkiye; 12Hospital de Pediatría Garrahan, Buenos Aires, Argentina; 13Universitätsklinikum Dresden, Dresden, Germany; 14University Children’s Hospital Krakow, Krakow, Poland; 15Uniersity of Sheffield, Sheffield, UK; 16University of Augsburg, Augsburg, Germany; 17St. Jude Children’s Research Hospital, Memphis, USA; 18All India Institute of Medical Sciences, New Delhi, India; 19Federal University of Paraná, Parana, Brazil; 20National Institutes of Health, Bethesday, USA; 21Regina Margherita Children’s Hospital, Turin, Italy; 22The University of Texas MD Anderson Cancer Center, Houston, USA; 23Justus Liebig University Giessen, Giessen, Germany
JOINT1366
Background: Paediatric Adrenocortical Tumours (pACTs) are potentially devastating neoplasms of the adrenal cortex. The incidence of pACTs is about 0.2–0.3 cases per million children per year in most countries. Most pACTs are hormonally active and most often present with androgen excess. Data suggest that pACTs that solely produce androgens have better survival than tumours that concomitantly produce other steroid classes. Although a thorough endocrine work-up is recommended, there is no consensus on how this should be performed, nor is there guidance on imaging modalities or the extent of genetic investigations.
Aim: To seek international consensus on the initial diagnostic work-up in children and young people with pACTs.
Methods: A modified three-step Delphi consensus method was applied to develop statements from an international group of pACT experts (Delphi panel), comprising 28 participants from Europe, the Americas, and Asia selected by their clinical merit and peer-reviewed publications in the field of pACTs. Electronic surveys were created by the steering committee (members of the European Network for the Study of Adrenal Tumours [ENS@T], KIDS working group) and circulated to the panel. Levels of agreement and disagreement were rated on a six-point Likert response scale with the opportunity to give feedback. After each round, results were discussed within the ENS@T-KIDS group, and questions were reformulated if no consensus (defined as agreement by at least 70%) was reached.
Results: Of the 28 voting members, 24 participated in round 1 (86%), 23 in voting round 2 (82%) and 24 in round 3 (86%). Based on a literature review, 45 statements were formulated by the steering committee and electronically distributed to the Delphi panel for voting. The statements cover four categories: (I) General Aspects and Clinical Assessment (17 statements); (II) Endocrine Work-up (including methodology and assessment of hormone excess; 15 statements); (III) Radiology (7 statements) and (IV) Genetics (6 statements). Of the 45 statements voted upon in round one, only eight did not reach consensus. These eight statements were revised and voted upon in round two, with seven reaching consensus. One statement was further revised based on panel/committee feedback and reached a consensus on round three.
Discussion and conclusion: The diagnostic work-up of rare pACTs varies amongst different centres and countries and partly depends on local resources and individual experiences. For the first time, we have developed expert-based consensus statements formulating a diagnostic pathway to guide a unified approach with practical considerations for the work-up of pACTs.
Volume 110
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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