Primary Intradural Extramedullary Sporadic Spinal Hemangioblastomas: Case Report and Systematic Review

BACKGROUND: Hemangioblastomas are benign vascular neoplasms of the central nervous system that may occur sporadically or in association with Von Hippel-Lindau disease. A minority of these lesions can occur in the spine, mostly as intramedullary masses. The authors present a rare case of primary sporadic spinal hemangioblastoma occurring as an intradural extramedullary (IDEM) lesion. Diagnostic workup and surgical management of the patient are described. METHODS: A systematic MEDLINE search was conducted using the keywords "hemangioblastoma" and "intradural extramedullary," "extramedullary," or "cauda equina." Clinicopathological characteristics and outcomes of the present case were reviewed and compared with those in the literature. RESULTS: A 72-year-old man was found to have an IDEM lesion in his cervical spine after presenting with neck and shoulder pain. Gross total resection was successfully performed with sacrifice of an involved dorsal nerve rootlet. Screening for Von Hippel-Lindau was negative. Thirty-three additional patients with sporadic IDEM hemangioblastomas are reported in the literature. There was a slight male preponderance (54%) with a median age of 52 years. Patients presented with pain (54%), radiculopathy (33%), or myelopathy (32%). The majority of lesions were located in the lumbosacral spine (56%). All patients underwent maximal safe resection with stable or improved clinical status. CONCLUSION: Primary IDEM hemangioblastomas are a rare entity. Differential diagnosis includes other IDEM lesions, such as schwannomas, meningiomas, or some vascular malformations. Resection of these sporadic tumors can be safely performed and result in improvement of neurologic deficits associated with mass effect from the tumor with low likelihood of recurrence.