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Review
. 2024 Feb;45(1):28-34.
doi: 10.1007/s00292-023-01289-z. Epub 2024 Jan 5.

[Multiple neuroendocrine tumors of the pancreas]

[Article in German]
Bence Sipos  1   2
Affiliations
Review

[Multiple neuroendocrine tumors of the pancreas]

[Article in German]
Bence Sipos. Pathologie (Heidelb). 2024 Feb.

Abstract
in English, German

Multiple neuroendocrine tumors (NET) of the pancreas often have a hereditary background. Sporadic and hereditary NET do not differ morphologically or with regard to their hormone expression. The most important clues for a hereditary background are provided by examination of the peritumoral pancreatic tissue, especially the morphology and hormone expression of the endocrine islets. Hyperplastic or dysplastic islets and microtumors with aberrant distribution of insulin and glucagon are the main features of hereditary NET. Morphological diagnosis of potentially hereditary NET has a relevant impact on the prognosis and clinical care of patients.

Multiple neuroendokrine Tumoren (NET) des Pankreas haben häufig einen hereditären Hintergrund. Sporadische und hereditäre NET unterscheiden sich weder morphologisch noch hinsichtlich ihrer Hormonexpression voneinander. Die wichtigsten Hinweise für einen hereditären Hintergrund liefert die Untersuchung des peritumoralen Pankreasgewebes, insbesondere der Morphologie und Hormonexpression der endokrinen Inseln. Hyperplastische oder dysplastische Inseln, Mikrotumoren mit aberranter Verteilung von Insulin, Glukagon sind die wichtigsten Merkmale von hereditären NET. Die morphologische Verdachtsdiagnose auf hereditäre NET hat einen relevanten Einfluss auf die Prognose und klinische Versorgung der Patienten.

Keywords: GCHN; Hereditary; MEN1; Neoplasia; VHL.

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