1. Hemangioblastoma of the Peripheral Nervous System: A Critical Analysis of Its Rarity in Von Hippel-Lindau Disease
- Author
-
Nikhil K. Murthy, Robert J. Spinner, and Russell R. Lonser
- Subjects
Pathology ,medicine.medical_specialty ,von Hippel-Lindau Disease ,endocrine system diseases ,Tumor suppressor gene ,Central nervous system ,Disease ,urologic and male genital diseases ,Germline mutation ,Peripheral Nervous System Neoplasms ,Hemangioblastoma ,medicine ,Humans ,Von Hippel–Lindau disease ,Cerebellar Neoplasms ,neoplasms ,business.industry ,medicine.disease ,female genital diseases and pregnancy complications ,Multisystem disease ,medicine.anatomical_structure ,Peripheral nervous system ,Surgery ,Neurology (clinical) ,business - 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.
- Published
- 2021