European Standard Clinical Practice Guideline and EXPeRT Recommendations for the Diagnosis and Management of Gastroenteropancreatic Neuroendocrine Neoplasms in Children and Adolescents
Pediatric gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) are extremely rare and clinically heterogeneous. Management has largely been extrapolated from adult practice. This European Standard Clinical Practice Guideline (ESCP), developed by the EXPeRT network in collaboration with adult NEN experts, provides (adult) evidence-informed and consensus-driven recommendations. Using a literature review and GRADE methodology, we address clinical evaluation, imaging pathology, genetics, surgical and systemic therapies, and long-term surveillance. Surgical resection is the cornerstone in localized disease. Systemic treatments are largely adapted from adult data, including somatostatin analogues, radiopharmaceutical therapy, and targeted agents. Multidisciplinary care, genetic counseling, and lifelong follow-up are essential to optimize outcomes.
Authors
- Yves Réguerre (ORCID: https://orcid.org/0000-0001-7192-046X)
- Antje Redlich (ORCID: https://orcid.org/0000-0002-1732-1869)
- S. Hescot (ORCID: https://orcid.org/0000-0002-0869-3003)
- A. Ferrari
- Bİlgehan YalçIn
- Rainer Claus (ORCID: https://orcid.org/0000-0003-2617-8766)
- Gianni Bisogno (ORCID: https://orcid.org/0000-0003-4462-5523)
- Ewa Bień (ORCID: https://orcid.org/0000-0002-2091-2686)
- Małgorzata Krawczyk (ORCID: https://orcid.org/0000-0001-7093-8975)
- Michael Abele (ORCID: https://orcid.org/0000-0002-9780-603X)
- Jörg Fuchs (ORCID: https://orcid.org/0000-0001-6145-2391)
- C. Rigaud
- D. Orbach
- Calogero Virgone
- Ines B. Brecht
- Julien Hadoux
- Dominik T. Schneider
- Michaela Kuhlen
- Ricardo Lopez‐Almaraz
- Tal Ben‐Ami
- Constantin Lapa
- Christian Vokuhl
- Maja Mazic Cesen
- Thomas Walter
Institutions
- University of Padua (IT)
- Technische Hochschule Augsburg (DE)
- Witten/Herdecke University (DE)
- University of Augsburg (DE)
- University of Milan (IT)
- University of Reunion Island (RE)
- Université Paris Sciences et Lettres (FR)
- Université Paris-Saclay (FR)
- University Hospital Bonn (DE)
- Hadassah Medical Center (IL)
- Kaplan Medical Center (IL)
- Ljubljana University Medical Centre (SI)
- Institut Gustave Roussy (FR)
- BioCruces Health research Institute (ES)
- Hospices Civils de Lyon (FR)
- University Children's Hospital Tübingen (DE)
- Klinikum Dortmund (DE)
- KUKA (Germany) (DE)
- Hospital de Cruces (ES)
- Fondazione IRCCS Istituto Nazionale dei Tumori (IT)
- Hacettepe University (TR)
- Gdańsk Medical University (PL)
- Institut Curie (FR)
- Otto-von-Guericke-Universität Magdeburg (DE)
Publication Details
- Journal
- Pediatric Blood & Cancer
- Published
- 2026-03-15
- DOI
- https://doi.org/10.1002/1545-5017.70194
- Primary Topic
- Neuroendocrine Tumor Research Advances
- Type
- article
- Field-Weighted Citation Impact
- 0.00