ArticleBritish journal of cancer2017
Childhood neuroendocrine tumours: a descriptive study revealing clues for genetic predisposition.
Article in British journal of cancer, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 9 papers.
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Who cites it
9 citing papers in PubMed, 30 citations in OpenAlex.
- Safety and dosimetry of [European journal of nuclear medicine and molecular imaging · 2025Trial
- Gastroenteropancreatic neuroendocrine neoplasms in children and adults: a comparative review.Frontiers in endocrinology · 2025Review
- Pediatric cancer predisposition syndromes involving non-central nervous system solid pediatric tumors: a review on their manifestations with a focus on histopathology.Virchows Archiv : an international journal of pathology · 2025Review
- Fortuitous discovery of an early neuroendocrine tumor during appendicular peritonitis.Annals of medicine and surgery (2012) · 2022Article
- Unique Finding of a Primary Central Nervous System Neuroendocrine Carcinoma in a 5-Year-Old Child: A Case Report.Frontiers in neuroscience · 2022Article
- Hypoglycemia secondary to insulinoma masking the onset of type 1 diabetes in an adolescent.Clinical case reports · 2021Article
- Pancreatic VIPoma as a Differential Diagnosis in Chronic Pediatric Diarrhea: A Case Report and Review of the Literature.Journal of medical cases · 2021Article
- Neuroendocrine Tumors in Pediatrics.Global pediatric health · 2019Review
- A 17-year-old male with a Small Bowel Neuroendocrine Tumor: flushing differential diagnosis.The World Allergy Organization journal · 2017Article
Corrections and comments
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Authors and funding
7 authors at 5 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundNeuroendocrine tumours (NETs) are rare in children and limited data are available. We aimed to specify tumour and patient characteristics and to investigate the role of genetic predisposition in the aetiology of paediatric NETs.
methodsUsing the Dutch Pathology Registry PALGA, we collected patient- and tumour data of paediatric NETs in the Netherlands between 1991 and 2013 (N=483).
resultsThe incidence of paediatric NETs in the Netherlands is 5.40 per one million per year. The majority of NETs were appendiceal tumours (N=441;91.3%). Additional surgery in appendiceal NETs was indicated in 89 patients, but performed in only 27 of these patients. Four out of five patients with pancreatic NETs were diagnosed with Von Hippel-Lindau disease (N=2) and Multiple Endocrine Neoplasia type 1 (N=2). In one patient with an appendiceal NET Familial Adenomatous Polyposis was diagnosed. On the basis of second primary tumours or other additional diagnoses, involvement of genetic predisposition was suggestive in several others.
conclusionsWe identified a significant number of patients with a confirmed or suspected tumour predisposition syndrome and show that paediatric pancreatic NETs in particular are associated with genetic syndromes. In addition, we conclude that treatment guidelines for appendiceal paediatric NETs need revision and improved implementation.
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