Surgical Management of Hemangioblastoma of the Spinal Cord: A Report of 18 Cases

Abstract

The cases of 18 patients operated on for hemangioblastoma of the spinal cord were reviewed. There were 11 men and 7 women. (M:F = 1.6:1) and in 15 patients, the onset of spinal symptoms was before the age of 40 years. There were 19 intradural and 2 extradural tumors. The tumor was accompanied by a cyst in 9 patients (50%). There were 6 cases of Lindau's disease, including 3 cases of multiple spinal tumors. Symptoms improved postoperatively in 13 patients, were unchanged in 2, and were aggravated in 3. At the present time, magnetic resonance imaging with gadolinium-diethylene-triamine-pentaacetic acid (Gd-DTPA) enhancement is the most useful tool for precise localization of the tumor and differentiation of any accompanying cyst.

Spinal Cord Hemangioblastoma : Diagnosis and Clinical Outcome after Surgical Treatment

“…A difference in neurological symptom was not observed between patients harboring sporadic hemangioblastomas and those harboring hemangioblastomas as a manifestation of VHL disease. Regards to the location of the tumor, intramedullary hemangioblastomas were reported as rare cases in previous review 4,18,20,26,28,30,32,[34][35][36] , but in our study intramedullary location (66%) was the most common.…”

“…Hemangioblastomas are vascular tumors that can be found throughout the central nervous system including spinal cord that account for approximately 2% of primary spinal cord tumors 9,18,35) . They are benign neoplasm (World Health Organization Grade I), but can cause significant morbidity and mortality through mass effect according to the intraspinal lesion 20,27) .…”

“…Von Hippel-Lindau (VHL) disease denotes an important subset of patients who present with hemangioblastomas in the central nervous system (CNS). Hemangioblastomas of the spinal cord occur more commonly as sporadic isolated lesions (70-80% of cases) rather than as multiple lesions in the cerebellum and retina as part of the VHL disease 1,7,15,[18][19][20]35) . The basis of familial inheritance of VHL disease is a germline mutation in the VHL tumor suppressor gene, which is located in the short arm of chromosome 3p25 10) .…”

Spinal cord hemangioblastomas in von Hippel–Lindau disease

“…This multiplicity accounts for the varied natural history of spinal cord hemangioblastomas associated with VHL, complexities in their management and uncertainties associated with longterm functional outcome. 14,18,19 Our study revealed that 66 (46.5%) out of 142 VHL patients examined had spinal cord hemangioblastomas. Although sporadic spinal cord hemangioblastomas are found in approximately 10-20% among all CNS hemangioblastomas, spinal cord hemangioblastoma with VHL is a more common manifestation.…”

“…15,18,19 Spinal cord hemangioblastomas may occur sporadically or in association with VHL, which is a multisystem familial cancer syndrome. Approximately two-thirds are sporadic in origin and the other third are associated with VHL.…”