Surgical management of lumbosacral nerve root hemangioblastomas in von Hippel-Lindau syndrome

Russell R. Lonser, Scott D. Wait, John A. Butman, Alexander Vortmeyer, McClellan M. Walther, Lance S. Governale, Edward H. Oldfield

Research output: Contribution to journalArticle

33 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)64-69
Number of pages6
JournalJournal of neurosurgery
Volume99
Issue number1 SUPPL.
StatePublished - Jul 1 2003
Externally publishedYes

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Hemangioblastoma
von Hippel-Lindau Disease
Neoplasms
Central Nervous System
Lumbosacral Region
Tumor Burden
Magnetic Resonance Imaging

Keywords

  • Hemangioblastoma
  • Microsurgery
  • Neoplasm
  • Nerve root
  • Von Hippel-Lindau syndrome

ASJC Scopus subject areas

  • Surgery
  • Clinical Neurology

Cite this

Lonser, R. R., Wait, S. D., Butman, J. A., Vortmeyer, A., 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.), 64-69.

Surgical management of lumbosacral nerve root hemangioblastomas in von Hippel-Lindau syndrome. / Lonser, Russell R.; Wait, Scott D.; Butman, John A.; Vortmeyer, Alexander; Walther, McClellan M.; Governale, Lance S.; Oldfield, Edward H.

In: Journal of neurosurgery, Vol. 99, No. 1 SUPPL., 01.07.2003, p. 64-69.

Research output: Contribution to journalArticle

Lonser, RR, Wait, SD, Butman, JA, Vortmeyer, A, Walther, MM, Governale, LS & Oldfield, EH 2003, 'Surgical management of lumbosacral nerve root hemangioblastomas in von Hippel-Lindau syndrome', Journal of neurosurgery, vol. 99, no. 1 SUPPL., pp. 64-69.
Lonser RR, Wait SD, Butman JA, Vortmeyer A, Walther MM, Governale LS et al. Surgical management of lumbosacral nerve root hemangioblastomas in von Hippel-Lindau syndrome. Journal of neurosurgery. 2003 Jul 1;99(1 SUPPL.):64-69.
Lonser, Russell R. ; Wait, Scott D. ; Butman, John A. ; Vortmeyer, Alexander ; Walther, McClellan M. ; Governale, Lance S. ; Oldfield, Edward H. / Surgical management of lumbosacral nerve root hemangioblastomas in von Hippel-Lindau syndrome. In: Journal of neurosurgery. 2003 ; Vol. 99, No. 1 SUPPL. pp. 64-69.
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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.",
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AU - Walther, McClellan M.

AU - Governale, Lance S.

AU - Oldfield, Edward H.

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