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

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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.

Article Information

Address reprint requests to: Russell R. Lonser, M.D., Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, 10 Center Drive, Building 10, Room 5D37, Bethesda, MD 20892–1414. email: lonserr@ninds.nih.gov.

© AANS, except where prohibited by US copyright law.

Headings

Figures

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    Representative intraoperative photograph demonstrating a small L-3 nerve root hemangioblastoma. The tumor envelops and displaces the fascicles of the involved nerve root. A large feeding vessel courses along the nerve root (arrowheads).

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    Case 6. Photomicrographs. Left: Hematoxylin and eosin staining demonstrating the classic features of a hemangioblastoma. These include a highly vascular capillary plexus surrounded by morphologically bland, vacuolated stromal cells. Multiple nerve root fascicles can be seen within the hemangioblastoma. Right: Brown reaction product staining showing S100-positive nerve root fascicles (arrows) within the hemangioblastoma. Tumor cells also stained positive for the S100 antigen. Original magnification × 40.

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