Az endokrin pancreas öröklodo daganatai

Translated title of the contribution: Hereditary tumours of the endocrine pancreas

Péter Igaz, Iván Igaz, Károly Ráz, Zsolt Tulassay

Research output: Contribution to journalReview article

2 Citations (Scopus)

Abstract

The pathogenesis, diagnosis and therapy of tumours originating from the endocrine pancreas represent one of the most exciting challenges of contemporary medicine. Some of these tumours appear as part of four hereditary syndromes (multiple endocrine neoplasia type 1 (MEN1), von Hippel-Lindau disease (VHL), neurofibromatosis type 1 and tuberous sclerosis) that are all inherited as autosomal dominant traits and result from mutations of tumour suppressor genes. Considering its clinical relevance, MEN1 appears to be the most important among these four syndromes. Tumours of the endocrine pancreas develop in 30-80% of patients carrying mutations of the MEN1 gene. Gastrinomas are the most frequent functioning tumours in MEN1 patients, followed by insulinomas, whereas other tumors e.g. glucagonoma, VIP-oma, GRF-oma and somatostatinoma occur very rarely. Tumours of the endocrine pancreas are infrequent in patients suffering from VHL, neurofibromatosis and tuberous sclerosis. In this review article, the authors present a synopsis of tumours of the endocrine pancreas related to these hereditary syndromes underlining the clinical charasteristics, diagnostical and therapeutical possibilities.

Original languageHungarian
Pages (from-to)195-200
Number of pages6
JournalOrvosi hetilap
Volume147
Issue number5
Publication statusPublished - Feb 5 2006

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ASJC Scopus subject areas

  • Medicine(all)

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