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Von Hippel–Lindau Syndrome

  • Jie ZhouEmail author
  • Xiaoran Li
Chapter

Abstract

Von Hippel–Lindau syndrome (VHL) is a heritable neoplastic disease resulting in multisystem carcinoma, benign tumors, and cysts. VHL is inherited in an autosomal dominant manner and could result in benign or malignant tumors, with high penetrance affecting multiple organ systems, including the central nervous system (CNS) and visceral organs [1]. Incidences of VHL are associated with a germline mutation of a tumor suppressor gene, VHL, on the short arm of chromosome 3 (3p25.5) [2].

Keywords

Von Hippel–Lindau syndrome Hemangioblastoma Clear-cell renal cell carcinoma Pheochromocytoma Pancreatic neuroendocrine tumor and cystadenoma Endolymphatic sac tumor Epididymal papillary cystadenomas 

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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of AnesthesiologyBrigham and Women’s Hospital, Harvard Medical SchoolBostonUSA

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