European Standard Clinical Practice Guideline and EXPeRT Recommendations for the Diagnosis and Management of Gastroenteropancreatic Neuroendocrine Neoplasms in Children and Adolescents.

This publication presents a European Standard Clinical Practice Guideline for pediatric gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs), created by the EXPeRT pediatric network in collaboration with adult NEN experts.

Using literature review and GRADE methodology, the guideline offers evidence-informed and consensus-based recommendations for:

• Clinical evaluation and imaging – Structured assessment and appropriate imaging strategies for diagnosis and staging.
• Pathology and genetics – Standardized pathologic characterization and integration of genetic counseling/testing, reflecting the inherited predisposition seen in a subset of patients.
• Treatment of localized disease – Surgical resection is emphasized as the primary and central treatment modality when cure is possible.
• Systemic therapies for advanced disease – Strategies are largely adapted from adult NEN management, including somatostatin analogues, radiopharmaceutical therapies, and targeted agents, recognizing the limited pediatric-specific evidence.
• Long-term surveillance – Lifelong, structured follow-up is recommended to detect recurrence, manage late effects, and address endocrine and growth-related issues.
• Care organization – The guideline underscores the necessity of multidisciplinary management in specialized centers, with close integration of pediatric and adult NEN expertise.

Overall, it provides a framework to standardize and optimize the care of children and adolescents with GEP-NENs despite the scarcity of pediatric-specific data.