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


Kuhlen M., Virgone C., Redlich A., Abele M., Lopez-Almaraz R., Ben-Ami T., ...Daha Fazla

Pediatric Blood and Cancer, 2026 (SCI-Expanded, Scopus)

  • Yayın Türü: Makale / Tam Makale
  • Basım Tarihi: 2026
  • Doi Numarası: 10.1002/1545-5017.70194
  • Dergi Adı: Pediatric Blood and Cancer
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, BIOSIS, CINAHL, EMBASE, MEDLINE
  • Anahtar Kelimeler: clinical guidance, EXPeRT, gastroenteropancreatic, neuroendocrine neoplasms, pediatric GEP-NEN, rare tumors
  • Hacettepe Üniversitesi Adresli: Evet

Özet

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.