Article
Pancreatic endocrine microadenomatosis in patients with von Hippel-Lindau disease: characterization by VHL/HIF pathway proteins expression.
The American journal of surgical pathology - 1 May 2009
Périgny Martine, Hammel Pascal, Corcos Olivier, Larochelle Olivier, Giraud Sophie, Richard Stéphane, Sauvanet Alain, Belghiti Jacques, Ruszniewski Philippe, Bedossa Pierre, Couvelard Anne
Abstract excerpt
INTRODUCTION: Von Hippel-Lindau (VHL) disease is an inherited syndrome caused by germline mutation in the VHL tumor suppressor gene predisposing to pancreatic endocrine tumors (PET). Whether these tumors derive from preexisting endocrine microadenomatosis as in multiple endocrine neoplasia type 1 (MEN1) is yet unknown. pVHL regulates hypoxia-inducible factor (HIF) that causes transcription activity of target...
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