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 significantly associated with the presence of distant metastases (M0 vs. M1, p = .0082) and complete surgical resection (R₀ vs. R_1/2 vs. no resection, p = .0077) but not with lymph node involvement (N0 vs. N1, p = .12), tumor localization within the pancreas (head vs. body vs. tail, p = .86), the extent of the primary tumor (pT1-2 vs. pT3-4, p = 1.0), pathological grade (G1 vs. G2-3, p = .28), or proliferation index (Ki67 ≤ 10% vs. >10%, p = .11). This study underscores the importance of disease stage and surgical resection as key prognostic factors in pediatric panNETs. It highlights the need for pediatric-specific management guidelines, integration of genetic screening, and expanded molecular profiling to optimize outcomes for children and adolescents with panNETs.