Pediatric neuroendocrine tumors in Denmark: Incidence, management, and
outcome from 1995–2020
Abstract
Background Neuroendocrine tumors (NETs), although rare, are
considered one of the most common gastrointestinal and bronchopulmonary
pediatric neoplasms. We aimed to determine the incidence, tumor
characteristics, management, and outcome of NETs and explore the role of
genetic predisposition in a Danish pediatric population.
Methods Using the Danish National Pathology Registry, we
conducted a nationwide retrospective study including all Danish children
aged ≤18 years diagnosed with a pathology-proven NET between 1995 and
2020. Results We identified 220 patients, with a 1.89:1 girl to
boy ratio. The yearly incidence for Danish pediatric NETs was 6.84 per 1
million children, with no significant change in incidence throughout the
observation period. NETs were located in the appendix (93.2%), the
pulmonary system (4.5%) and pancreas (2.3%). One recurrence was noted
in the pancreas in a genetically predisposed patient with multiple
neuroendocrine neoplasia type 1 (MEN1), resulting in an overall
recurrence rate of 0.5% (0% in appendiceal NETs; 0% in
bronchopulmonary NETs; 20.0% in pancreatic NETs). No NET-related
mortality was registered. Four patients had a known predisposing genetic
condition, one appendiceal NET and neurofibromatosis type 1, and three
pancreatic NETs associated with MEN1. Postsurgical surveillance regimes,
choice of tumor markers, and imaging modality varied throughout the
study period. Conclusions We confirmed a stable incidence of
pediatric NETs during the study period. The overall recurrence rate was
0.5% and no NET-related mortality was observed. Known genetic
predisposition was present in 1.8% of patients. Future guidelines
should consider the apparent indolent nature and excellent prognosis of
these tumors.