Abstract

Von Hippel-Lindau (VHL) syndrome is a rare hereditary disorder characterized by the development of multiple tumorsin various organs. This paper provides a comprehensive overview of the VHL gene, its mechanism of action, and itsimplications in VHL-related tumorigenesis. The first section explores the role of the VHL tumor suppressor gene inregulating angiogenesis and oxygen sensing, highlighting its significance in tumor suppression. The second sectiondiscusses the association between VHL gene mutations and the development of various tumor types, emphasizing thediverse clinical manifestations of VHL syndrome. The third section examines the diagnostic methods for identifyingVHL gene mutations, including genetic testing and clinical evaluations. The fourth section delves into therapeuticapproaches for managing VHL-related tumors, encompassing surgical interventions, targeted therapies, radiotherapy,and emerging treatment strategies. This paper aims to enhance understanding of VHL syndrome, facilitate earlydiagnosis, and guide treatment decisions for individuals affected by this complex disorder.

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