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Published on: October 20, 2017
Management of von Hippel-Lindau disease-associated CNS lesions
Joshua J Wind1, Russell R Lonser
1Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, NIH, Bethesda, MD, USA.
Insights
Von Hippel-Lindau (VHL) disease patients frequently develop CNS hemangioblastomas and endolymphatic sac tumors (ELSTs). Optimizing surgical management and perioperative care improves outcomes and reduces morbidity for these VHL-associated tumors.
Area of Science:
- Neurosurgery
- Oncology
- Genetics
Background:
- Von Hippel-Lindau (VHL) disease is a genetic disorder associated with a high incidence of central nervous system (CNS) tumors, primarily hemangioblastomas and endolymphatic sac tumors (ELSTs).
- Hemangioblastomas affect 60-80% of VHL patients, while ELSTs occur in 10-15%.
Purpose of the Study:
- To review the perioperative management, surgical indications, operative principles, and outcomes for VHL-associated hemangioblastomas and ELSTs.
- To highlight strategies for optimizing surgical interventions to minimize patient morbidity.
Main Methods:
- Review of current literature on the surgical management of VHL-associated tumors.
- Analysis of perioperative considerations, surgical techniques, and patient outcomes.
Main Results:
- Advances in understanding VHL natural history and surgical techniques have improved patient management.
- Careful patient selection and refined surgical approaches are crucial for reducing morbidity.
Conclusions:
- Optimizing surgical indications and techniques for CNS hemangioblastomas and ELSTs in VHL patients is essential.
- Improved perioperative management and surgical strategies lead to better outcomes for individuals with VHL disease.
Abstract:
Patients with von Hippel-Lindau disease (VHL) often harbor significant disease burden within the CNS, specifically craniospinal-axis hemangioblastomas and endolymphatic sac tumors (ELSTs). The majority (60-80%) of patients with VHL harbor hemangioblastomas, and 10-15% will develop ELSTs. Advances in the understanding of the natural history and outcomes associated with the surgical management of VHL-associated tumors have led to improved management of patients with VHL. Optimizing indications for surgical intervention and refining of surgical techniques for these lesions can reduce patient morbidity associated with the management of this syndrome. In this article, we review the various aspects of perioperative management of patients with VHL, surgical indications and general operative principles for the management of hemangioblastomas and ELSTs, and outcomes associated with the surgical treatment of these tumors.
