Evidence map›Paper›PMID 41647110›Full record

ReviewFrontiers in endocrinology2025

Gastroenteropancreatic neuroendocrine neoplasms in children and adults: a comparative review.

Michaela Kuhlen, Rainer Claus, Marianne E Pavel, Marina Kunstreich, Nehara Begum, Constantin Lapa, Antje Redlich

Abstract readReviewComparative Study
In one paragraph

Review in Frontiers in endocrinology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

0numbers the graph read from it
0cells of the map it votes in
1citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

1 citing paper in PubMed.

  1. Review
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

7 authors.

Michaela KuhlenPediatrics and Adolescent Medicine, Faculty of Medicine, University of Augsburg, Augsburg, Germany.
Rainer ClausBavarian Cancer Research Center (BZKF), Augsburg, Germany.
Marianne E PavelDepartment of Medicine 1, Uniklinikum Erlangen and Comprehensive Cancer Center CCC-EMN, Friedrich Alexander University Erlangen-Nürnberg, Erlangen, Germany.
Marina KunstreichPediatrics and Adolescent Medicine, Faculty of Medicine, University of Augsburg, Augsburg, Germany.
Nehara BegumDepartment for General-, Visceral-, Thoracic- and Endocrine Surgery, European Neuroendocrine Tumor Society (ENETS)-Center of Excellence, Johannes-Wesling-Klinikum Minden, University Hospital of the Ruhr-University Bochum, Minden, Germany.
Constantin LapaNuclear Medicine, Faculty of Medicine, University of Augsburg, Augsburg, Germany.
Antje RedlichDepartment of Pediatrics, Pediatric Hematology/Oncology, Otto-von-Guericke-University, Magdeburg, Germany.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Background: Gastroenteropancreatic neuroendocrine neoplasms (GEPNENs) represent a biologically heterogeneous tumor group that is increasingly recognized in adults but remains exceptionally rare in children. While adult management is guided by evidence-based recommendations, pediatric practice relies mainly on registry data and extrapolation. A direct comparison is needed to identify shared principles, highlight divergences, and define research priorities. Methods: We performed a structured literature review of pediatric GEPNENs (pancreatic, gastrointestinal [excluding appendix], and neuroendocrine neoplasms of unknown primary) and contrasted these findings with adult guidelines (ENETS 2023-2024, ESMO 2020-2024, ASCO 2023, NANETS 2018-2023) and pivotal clinical trials. Domains analyzed included epidemiology, clinical presentation, histological and molecular characteristics, treatment strategies, outcomes, and guideline frameworks. Results: Pediatric GEPNENs are strongly enriched for hereditary cancer predisposition syndromes (MEN1, VHL, NF1, TSC) and show a predominance of well-differentiated NET G1-G2. In contrast, adults exhibit the full spectrum of NET G1-3 and NEC G3. Somatostatin receptor (SSTR) expression is frequent in both pediatric and adult NETs, supporting the use of somatostatin analogues (SSAs) and peptide receptor radionuclide therapy (PRRT) in advanced disease; SSTR expression declines with increasing grade. Surgical resection remains the only curative option in both populations, with pediatric practice prioritizing organ preservation and minimization of late effects. In adults, systemic therapy sequencing is structured by randomized trials, whereas pediatric use of systemic therapies is adapted case-by-case, with emerging but still limited evidence. Survival in localized pediatric NETs exceeds 90%, but remains poor in metastatic and high-grade disease, similar to adults. Conclusions: Although histological frameworks are shared, pediatric GEPNENs differ from adult disease in genetics, site distribution, functional status, and survivorship challenges. Adult evidence may be cautiously adapted to pediatrics, but pediatric-specific guidelines and collaborative research are urgently needed to address unique biological and clinical features and to harmonize long-term care.

Indexed as

Intestinal NeoplasmsNeuroendocrine TumorsPancreatic NeoplasmsStomach NeoplasmsAdultChildHumansadultschildren and adolescentsgastroenteropancreaticmanagementneuroendocrine neoplasms

Identifiers

PMID41647110
PMCPMC12867895

What OpenQuestion holds

Textmetadata
LicenceCC BY
Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.