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Neuroendocrine tumors (NETs) are neoplasms that arise from cells of the endocrine (hormonal) and nervous systems. Many are benign, while some are malignant. They most commonly occur in the intestine, where they are often called carcinoid tumors, but they are also found in the pancreas, lung and the rest of the body.
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abstract:
Childhood neuroendocrine tumours: a descriptive study revealing clues for genetic predisposition : British Journal of Cancer
Neuroendocrine 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.
Methods:
Using
the Dutch Pathology Registry PALGA, we collected patient- and tumour
data of paediatric NETs in the Netherlands between 1991 and 2013 (N=483).
Results:
The
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.
Conclusions:
We
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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