Hemangioblastoma of the Peripheral Nervous System: A Critical Analysis of Its Rarity in Von Hippel-Lindau Disease.
Where this comes from
- Record sourced from PubMed, PMID 34343683.
- Also identified by DOI 10.1016/j.wneu.2021.07.123.
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Abstract
Von Hippel-Lindau (VHL) disease is a multisystem disease cause by germline mutations of the VHL tumor suppressor gene. Hemangioblastomas are the most common manifestation of VHL disease and can occur in the central nervous system in up to 90% of these patients. By contrast, we found only one true case of a peripheral nervous system (PNS) hemangioblastoma in the setting of VHL. We explore the possible reasons behind the lack of PNS hemangioblastomas in VHL disease.
Medical subject headings
- Cerebellar Neoplasms
- Hemangioblastoma
- Peripheral Nervous System Neoplasms
- von Hippel-Lindau Disease