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[Hereditary syndromes of neuroendocrine tumours].

Authors
  • Kaemmerer, D
  • Posorski, N
  • Hommann, M
  • Baum, R P
  • Hörsch, D
Type
Published Article
Journal
Zentralblatt für Chirurgie
Publication Date
Aug 01, 2014
Volume
139
Issue
4
Pages
415–427
Identifiers
DOI: 10.1055/s-0033-1350692
PMID: 24327486
Source
Medline
License
Unknown

Abstract

Diffuse localised neuroendocrinal cells represent the largest population of endocrinally active cells and can degenerate to malignant neuroendocrine tumours (NET). In this review the most important hereditary syndromes that predispose for endocrine and neuroendocrine tumours are presented and discussed. NET occur mainly as sporadic tumours. Current investigations on the pathogenesis of sporadic neuroendocrine tumours have revealed a close relationship between hereditary and sporadic neuroendocrine tumours. In the course of hereditary syndromes, such as multiple endocrine neoplasia, endocrine and neuroendocrine tumours as well as non-endocrine neoplasias can occur. In order to recognise these syndromes in good time a knowledge of the predisposing syndromes and their cardinal symptoms is essential. In this way not only individualised diagnosis and therapy can be planned but also an appropriate early management of first degree relatives can be initiated.

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