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Surgical management of lumbosacral nerve root hemangioblastomas in von Hippel-Lindau syndrome.
J Neurosurg. 2003 Jul; 99(1 Suppl):64-9.JN

Abstract

OBJECT

Hemangioblastomas in the lumbosacral region are rare, and the authors of prior reports have not defined the surgical management, histopathological features, or outcome in a group of patients after resection of these tumors. To identify features that will help guide the operative and clinical management of these lesions, the authors reviewed data obtained in a series of patients with von Hippel-Lindau syndrome who underwent resection of lumbosacral nerve root hemangioblastomas.

METHODS

Six consecutive patients (three men and three women; mean age at surgery 39 years [range 31-48 years]) who underwent operations for resection of lumbosacral nerve root hemangioblastomas were included in this study. The mean follow-up period was 23 months (range 6-45 months). Data derived from examination, hospital charts, operative findings, histopathological analysis, and magnetic resonance imaging were used to analyze surgical management and clinical outcome. The resected tumors were located in the lumbar (five cases) or sacral (one case) regions; the mean tumor size was 2728 mm3 (range 80-15,022 mm3). Consistent with central nervous system (CNS) regional variation of space available to accommodate the neural compressive effect of the hemangioblastoma size, the mean tumor volume (2728 mm3) of these symptomatic lesions was much larger than that of symptomatic hemangioblastomas resected in the other regions of the CNS. Histopathological examination showed infiltration of the associated nerve root by the hemangioblastoma in each case. In five of the six patients complete resection was achieved, and in one patient intradural exploration of two hemangioblastomas was performed, but resection was not achieved because of motor root involvement. In all cases involving complete resections the patients experienced symptomatic improvement.

CONCLUSIONS

Lumbosacral nerve root hemangioblastomas can be safely removed in most patients with von Hippel-Lindau syndrome. Generally, hemangioblastomas of the lumbosacral nerve roots should be resected when they become symptomatic. Because these neoplasms appear to originate from the nerve root, it is necessary to sacrifice the nerve root from which the hemangioblastoma originates to achieve complete resection.

Authors+Show Affiliations

Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892-1414, USA. lonserr@ninds.nih.govNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article
Review

Language

eng

PubMed ID

12859062

Citation

Lonser, Russell R., et al. "Surgical Management of Lumbosacral Nerve Root Hemangioblastomas in Von Hippel-Lindau Syndrome." Journal of Neurosurgery, vol. 99, no. 1 Suppl, 2003, pp. 64-9.
Lonser RR, Wait SD, Butman JA, et al. Surgical management of lumbosacral nerve root hemangioblastomas in von Hippel-Lindau syndrome. J Neurosurg. 2003;99(1 Suppl):64-9.
Lonser, R. R., Wait, S. D., Butman, J. A., Vortmeyer, A. O., Walther, M. M., Governale, L. S., & Oldfield, E. H. (2003). Surgical management of lumbosacral nerve root hemangioblastomas in von Hippel-Lindau syndrome. Journal of Neurosurgery, 99(1 Suppl), 64-9.
Lonser RR, et al. Surgical Management of Lumbosacral Nerve Root Hemangioblastomas in Von Hippel-Lindau Syndrome. J Neurosurg. 2003;99(1 Suppl):64-9. PubMed PMID: 12859062.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Surgical management of lumbosacral nerve root hemangioblastomas in von Hippel-Lindau syndrome. AU - Lonser,Russell R, AU - Wait,Scott D, AU - Butman,John A, AU - Vortmeyer,Alexander O, AU - Walther,McClellan M, AU - Governale,Lance S, AU - Oldfield,Edward H, PY - 2003/7/16/pubmed PY - 2003/8/9/medline PY - 2003/7/16/entrez SP - 64 EP - 9 JF - Journal of neurosurgery JO - J Neurosurg VL - 99 IS - 1 Suppl N2 - OBJECT: Hemangioblastomas in the lumbosacral region are rare, and the authors of prior reports have not defined the surgical management, histopathological features, or outcome in a group of patients after resection of these tumors. To identify features that will help guide the operative and clinical management of these lesions, the authors reviewed data obtained in a series of patients with von Hippel-Lindau syndrome who underwent resection of lumbosacral nerve root hemangioblastomas. METHODS: Six consecutive patients (three men and three women; mean age at surgery 39 years [range 31-48 years]) who underwent operations for resection of lumbosacral nerve root hemangioblastomas were included in this study. The mean follow-up period was 23 months (range 6-45 months). Data derived from examination, hospital charts, operative findings, histopathological analysis, and magnetic resonance imaging were used to analyze surgical management and clinical outcome. The resected tumors were located in the lumbar (five cases) or sacral (one case) regions; the mean tumor size was 2728 mm3 (range 80-15,022 mm3). Consistent with central nervous system (CNS) regional variation of space available to accommodate the neural compressive effect of the hemangioblastoma size, the mean tumor volume (2728 mm3) of these symptomatic lesions was much larger than that of symptomatic hemangioblastomas resected in the other regions of the CNS. Histopathological examination showed infiltration of the associated nerve root by the hemangioblastoma in each case. In five of the six patients complete resection was achieved, and in one patient intradural exploration of two hemangioblastomas was performed, but resection was not achieved because of motor root involvement. In all cases involving complete resections the patients experienced symptomatic improvement. CONCLUSIONS: Lumbosacral nerve root hemangioblastomas can be safely removed in most patients with von Hippel-Lindau syndrome. Generally, hemangioblastomas of the lumbosacral nerve roots should be resected when they become symptomatic. Because these neoplasms appear to originate from the nerve root, it is necessary to sacrifice the nerve root from which the hemangioblastoma originates to achieve complete resection. SN - 0022-3085 UR - https://www.unboundmedicine.com/medline/citation/12859062/Surgical_management_of_lumbosacral_nerve_root_hemangioblastomas_in_von_Hippel_Lindau_syndrome_ L2 - https://thejns.org/doi/10.3171/spi.2003.99.1.0064 DB - PRIME DP - Unbound Medicine ER -