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
Kuhlen Kuhlen, Virgone Virgone, Redlich Redlich, Abele Abele, Lopez-Almaraz Lopez-Almaraz, Ben-Ami Ben-Ami, Bien Bien, Bisogno Bisogno, Cesen Cesen, Ferrari Ferrari, Krawczyk Krawczyk, Reguerre Reguerre, Schneider Schneider, Brecht Brecht, Rigaud Rigaud, Yalcin Yalcin, Claus Claus, Vokuhl Vokuhl, Lapa Lapa, Fuchs Fuchs, Hescot Hescot, Walter Walter, Hadoux Hadoux, Orbach Orbach
View on Pubmed