Functional Outcome After Resection of Von Hippel-Lindau Disease-Associated Cauda Equina Hemangioblastomas: An Observational Cohort Study.
Oper Neurosurg (Hagerstown). 2017 08 01; 13(4):435-440.ON

Abstract

BACKGROUND

Cauda equina hemangioblastomas in von Hippel-Lindau (VHL) disease can cause significant neurological signs and symptoms. Despite their associated morbidity, the management of these tumors remains incompletely defined.

OBJECTIVE

To determine optimal management, we analyzed the functional outcomes after resection of these tumors.

METHODS

VHL patients who underwent surgical resection of cauda equina hemangioblastomas at the National Institutes of Health and the University of Virginia were included. Clinical and radiological follow-up was performed at 6- to 12-month intervals after surgery.

RESULTS

Fifteen patients underwent 18 operations for 21 cauda equina hemangioblastomas (median follow-up 5.9 years). Patients often presented with multiple symptoms, including pain (67%), numbness (50%), urinary complaints (33%), and weakness (11%). Median preoperative tumor volume was 1.2 cm 3 . Four tumors at 3 operations were not resected due to a motor nerve root origin. Gross total resection was achieved in 14 surgeries (93% of operations when resection was attempted). New mild (non-function limiting) neurological symptoms were noted after 11 operations (61%), which most often (64%) resolved within 2 weeks of surgery. At 6-month follow-up, 15 patients (83%) were stable, 2 (11%) were improved, and 1 (6%) was worse. Histological analysis revealed that all tumors originated from within the involved nerve fascicle.

CONCLUSIONS

VHL-associated cauda equina hemangioblastomas have an intrafascicular origin and require interruption of the rootlet of origin for complete resection. Motor nerve root involvement may preclude complete resection but strategies including bony decompression and/or interruption of vascular supply may provide a therapeutic option. Nevertheless, most VHL patients with symptom-producing lesions improve with resection.

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Authors+Show Affiliations

Mehta GU
Surgical Neurology Branch, National Institutes of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland. Department of Neurosurgery, University of Virginia Health Sciences Center, University of Virginia, Charlottesville, Virginia.
Montgomery BK
Surgical Neurology Branch, National Institutes of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland.
Maggio DM
Surgical Neurology Branch, National Institutes of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland. Department of Neurosurgery, University of Virginia Health Sciences Center, University of Virginia, Charlottesville, Virginia.
Chittiboina P
Surgical Neurology Branch, National Institutes of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland.
Oldfield EH
Surgical Neurology Branch, National Institutes of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland. Department of Neurosurgery, University of Virginia Health Sciences Center, University of Virginia, Charlottesville, Virginia.
Lonser RR
Surgical Neurology Branch, National Institutes of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland. Department of Neurosurgery, Ohio State University Wexner Medical Center, Columbus, Ohio.

MeSH

AdultCauda EquinaCerebellar NeoplasmsCohort StudiesFemaleHemangioblastomaHumansLaminectomyMagnetic Resonance ImagingMaleMiddle AgedNeurologic ExaminationTomography Scanners, X-Ray ComputedTreatment OutcomeYoung Adultvon Hippel-Lindau Disease

Pub Type(s)

Journal Article
Observational Study
Research Support, N.I.H., Intramural

Language

eng

PubMed ID

28838111