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Pediatric neuroendocrine tumors in Denmark: Incidence, management, and outcome from 1995–2020
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  • Mona P. Ankerstjerne,
  • Sara Giovannoni,
  • Lene G. Christensen,
  • Sören Möller,
  • Pernille Holmager,
  • Ulrich Knigge,
  • Mark B. Ellebaek,
  • Mathias Rathe
Mona P. Ankerstjerne
Odense Universitetshospital

Corresponding Author:[email protected]

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Sara Giovannoni
Odense Universitetshospital Kirurgisk Afdeling A
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Lene G. Christensen
Odense Universitetshospital Afdeling for Klinisk Patologi
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Sören Möller
University of Southern Denmark
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Pernille Holmager
Rigshospitalet
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Ulrich Knigge
Rigshospitalet
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Mark B. Ellebaek
Odense Universitetshospital Kirurgisk Afdeling A
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Mathias Rathe
Odense Universitetshospital
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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.
03 Aug 2024Submitted to Pediatric Blood & Cancer
03 Aug 2024Submission Checks Completed
03 Aug 2024Assigned to Editor
05 Aug 2024Review(s) Completed, Editorial Evaluation Pending
13 Aug 2024Reviewer(s) Assigned
17 Sep 2024Editorial Decision: Revise Major
04 Oct 20241st Revision Received
04 Oct 2024Submission Checks Completed
04 Oct 2024Assigned to Editor
07 Oct 2024Review(s) Completed, Editorial Evaluation Pending
07 Oct 2024Reviewer(s) Assigned
16 Oct 2024Editorial Decision: Accept