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open access

Vol 71, No 3 (2020)
Review paper
Submitted: 2020-03-20
Accepted: 2020-03-22
Published online: 2020-06-30
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Pancreatic neuroendocrine tumours in patients with von Hippel-Lindau disease

Agnieszka Zwolak1, Joanna Świrska21, Ewa Tywanek21, Marta Dudzińska2, Jerzy S. Tarach1, Beata Matyjaszek-Matuszek1
DOI: 10.5603/EP.a2020.0027
·
Pubmed: 32797472
·
Endokrynol Pol 2020;71(3):256-259.
Affiliations
  1. Department of Endocrinology, Medical University in Lublin, Poland
  2. Chair of Internal Medicine and Department of Internal Nursing, Medical University in Lublin, Poland

open access

Vol 71, No 3 (2020)
Review Article
Submitted: 2020-03-20
Accepted: 2020-03-22
Published online: 2020-06-30

Abstract

Von Hippel-Lindau disease is a highly penetrant autosomal genetic disorder caused by a germline mutation in the tumour suppressor gene, manifesting with the formation of various tumours, including neuroendocrine tumours of the pancreas. The incidence of the latter is not very high, varying from 5% to 18%. To compare, haemangioblastomas and clear cell renal carcinoma are present in 70% of von Hippel-Lindau patients and are considered the main prognostic factors, with renal cancer being the most common cause of death. However, pancreatic neuroendocrine tumours should not be neglected, considering their malignant potential (different to sporadic cases), natural history, and treatment protocol. This paper aims to review the literature on the epidemiology, natural history, treatment, and surveillance of individuals affected by pancreatic neuroendocrine tumours in von Hippel-Lindau disease.

Abstract

Von Hippel-Lindau disease is a highly penetrant autosomal genetic disorder caused by a germline mutation in the tumour suppressor gene, manifesting with the formation of various tumours, including neuroendocrine tumours of the pancreas. The incidence of the latter is not very high, varying from 5% to 18%. To compare, haemangioblastomas and clear cell renal carcinoma are present in 70% of von Hippel-Lindau patients and are considered the main prognostic factors, with renal cancer being the most common cause of death. However, pancreatic neuroendocrine tumours should not be neglected, considering their malignant potential (different to sporadic cases), natural history, and treatment protocol. This paper aims to review the literature on the epidemiology, natural history, treatment, and surveillance of individuals affected by pancreatic neuroendocrine tumours in von Hippel-Lindau disease.

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Keywords

pancreatic neuroendocrine tumours; von Hippel-Lindau disease

About this article
Title

Pancreatic neuroendocrine tumours in patients with von Hippel-Lindau disease

Journal

Endokrynologia Polska

Issue

Vol 71, No 3 (2020)

Article type

Review paper

Pages

256-259

Published online

2020-06-30

Page views

1804

Article views/downloads

1883

DOI

10.5603/EP.a2020.0027

Pubmed

32797472

Bibliographic record

Endokrynol Pol 2020;71(3):256-259.

Keywords

pancreatic neuroendocrine tumours
von Hippel-Lindau disease

Authors

Agnieszka Zwolak
Joanna Świrska
Ewa Tywanek
Marta Dudzińska
Jerzy S. Tarach
Beata Matyjaszek-Matuszek

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