Pancreatic neuroendocrine tumors in children and adolescents-Data from the German MET studies (1997-2023).

ABSTRACT: Pancreatic neuroendocrine tumors (panNETs) are rare pediatric malignancies with age-specific clinical and biological features. Data on their presentation, management, and outcomes remain limited. This retrospective study analyzed 28 pediatric panNET cases from the German Malignant Endocrine Tumor (MET) Registry enrolled between 1997 and 2024. Clinical presentation, diagnostics, and treatment were evaluated to identify prognostic factors and outcomes. The cohort included 18 females (64.3%) and 10 males (35.7%), with a median age at diagnosis of 14.7 years. Nonfunctional tumors predominated (75%). Genetic syndromes were identified in 17.9% of patients. Localized disease showed a 3-year overall survival (OS) of 100%, while metastatic disease had a 3-year OS of 50.9%. Event-free survival was s

SUBMITTER: Karges K 

PROVIDER: S-EPMC12358202 | biostudies-literature | 2025 Aug

REPOSITORIES: biostudies-literature