Von Hippel-Lindau and Cancers
A special issue of Cancers (ISSN 2072-6694). This special issue belongs to the section "Molecular Cancer Biology".
Deadline for manuscript submissions: closed (15 November 2023) | Viewed by 1992
Special Issue Editors
Interests: adrenocortical cancer; neuroendocrine tumors; targeted therapy; drug discovery; drug delivery; nanomedicine; tumor microenvironment; tumor necrosis factor alpha
Interests: urologic oncologic surgery; renal cell carcinoma; hereditary kidney cancer syndromes; adrenal surgery; cancer genetics
Special Issue Information
Dear Colleagues,
Von Hippel–Lindau disease (VHL) is a rare and inheritable tumor syndrome with an autosomal dominant pattern that affects multiple organs. The disease was named after the German ophthalmologist, Eugen von Hippel, who first described ophthalmic angiomas in 1904, and the Swedish pathologist, Arvid Lindau, who discovered “angiomatosis of the central nervous system” in 1927. VHL disease is caused by germline alterations in the VHL gene. pVHL is a member of an E3 ubiquitin ligase complex. During normoxia, this complex ubiquitinates transcription factor hypoxia-inducible factor-alpha (HIF-α) for degradation by the proteasome. Alterations in VHL reduce the degradation of HIF, resulting in HIF accumulation and upregulation of multiple targets genes resulting in angiogenesis and tumorigenesis. The importance of this pathway was highlighted in 2019 as three investigators who discovered the cellular response to hypoxia received the Nobel Prize in Physiology or Medicine.
In this Special Issue, we include a brief history of VHL; the detailed molecular pathophysiology of VHL-HIF and tumorigenesis; organ-specific phenotypes such as retinal, brain, and spine hemangioblastomas; pheochromocytoma and paragangliomas; pancreatic cysts and neuroendocrine tumors; and urologic manifestations of VHL with a focus on renal cell cancer. The updated surgical and medical management and surveillance programs are also discussed.
Dr. Naris Nilubol
Dr. Mark W. Ball
Guest Editors
Manuscript Submission Information
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Keywords
- von Hippel–Lindau disease
- hemangioblastoma
- pheochromocytoma
- renal cell cancer
- pancreatic neuroendocrine tumor
- hypoxia-inducible factor
