Sporadic Intradural Extramedullary Hemangioblastoma of the Cauda Equina: Case Report and Literature Review.

BACKGROUND: Spinal hemangioblastomas account for 1%-5% of all spinal cord tumors. Although spinal hemangioblastomas are rare, it is exceedingly rare to have a case of intradural extramedullary hemangioblastoma of the spine, especially in isolation without von Hippel-Lindau syndrome. The purpose of this report is to present a rare case of intradural extramedullary hemangioblastoma of the cauda equina and a literature review. CASE DESCRIPTION: An 82-year-old man presented with an intradural mass at the L4 spinal level on magnetic resonance imaging. Given the benign appearance on magnetic resonance imaging and lack of radiculopathy, the decision was made to observe the patient. Over the course of 4 years, the tumor enlarged and caused left lower extremity radicular pain. Posterior laminectomy and tumor excision was performed. Owing to intimate involvement with multiple nerve roots, subtotal resection was performed. Immunohistopathologic analysis revealed a highly vascular and cellular tumor, with findings consistent with intradural extramedullary hemangioblastoma. The patient ultimately underwent CyberKnife radiosurgery for residual tumor, with improvement in neurologic symptoms. Follow-up studies at 2 years failed to show any tumor growth.
CONCLUSIONS: Literature review revealed cauda equina intradural extramedullary spinal hemangioblastoma to be a rare diagnosis. Furthermore, this was a sporadic case without associated von Hippel-Lindau syndrome. Only 20 previous cases have been reported. Immunohistopathologic analysis was required to make the diagnosis. Radiosurgery appears to be useful in cases of residual tumor after subtotal tumor resection.