Erschienen in:
26.04.2023 | Brief report
Gastrinomas and non-functioning pancreatic endocrine tumors in multiple endocrine neoplasia syndrome type-1 (MEN-1)
verfasst von:
Luigi Camera, Francesca Boccadifuoco, Roberta Modica, Luca Messerini, Antongiulio Faggiano, Valeria Romeo, Valeria Gaudieri, Annamaria Colao, Simone Maurea, Arturo Brunetti, on behalf of the European Neuroendocrine Tumor Society (ENETS) of Naples
Erschienen in:
Endocrine
|
Ausgabe 3/2023
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Excerpt
Multiple endocrine neoplasia type-1 (MEN-1) is a rare hereditary autosomal dominant syndrome due to frameshift and non-sense mutations in the MEN-1 tumor suppressor gene localized on the long arm of chromosome 11 [
1]. Also known as Wermer syndrome, it has a prevalence of 2–20/100,000 individuals who may develop multiple neoplastic lesions arising in the parathyroid (90–95%) as well as the pituitary glands (40–50%), the pancreatic islet cells (50–60%) and the duodenal wall (35–40%) [
2]. …