title: [Hereditary syndromes of neuroendocrine tumours].
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.
authors: Klinik für Allgemeine und Viszeralchirurgie, Zentralklinik Bad Berka GmbH, Bad Berka, Deutschland.
authors: D
authors: D
authors: Kaemmerer
authors: Institut für Humangenetik Core Unit Chip Applikationen, Universitätsklinikum Jena, Jena, Deutschland.
authors: N
authors: N
authors: Posorski
authors: Klinik für Allgemeine und Viszeralchirurgie, Zentralklinik Bad Berka GmbH, Bad Berka, Deutschland.
authors: M
authors: M
authors: Hommann
authors: Klinik für Molekulare Radiotherapie/Zentrum für Molekulare Bildgebung (PET/CT), Zentralklinik Bad Berka GmbH, Bad Berka, Deutschland.
authors: R P
authors: RP
authors: Baum
authors: Klinik für Innere Medizin/Gastroenterologie und Endokrinologie, Zentralklinik Bad Berka GmbH, Bad Berka, Deutschland.
authors: D
authors: D
authors: Hörsch
mesh_terms: D003937:Diagnosis, Differential; D005770:Gastrointestinal Neoplasms / Q000175:diagnosis / Q000235:genetics* / Q000601:surgery*; D006801:Humans; D018358:Neuroendocrine Tumors / Q000175:diagnosis / Q000235:genetics* / Q000601:surgery*; D013577:Syndrome