Microsurgical Treatment of Sporadic and von Hippel-Lindau Disease Associated Spinal Hemangioblastomas: A Single-Institution Experience

Abstract

Study DesignRetrospective cohort study.PurposeTo examine the clinical profile and surgical complications in patients with spinal hemangioblastomas and to evaluate the long-term outcome in them.Overview of LiteratureAlthough considered to be histologically benign, hemangioblastomas may cause significant neurological deficits. The proportion of spinal hemangioblastomas associated with von Hippel–Lindau (VHL) disease has been estimated be 13%–59%. Preoperative neurological function correlates with postoperative neurological status. Studies have shown no difference in outcomes between sporadic and VHL-associated spinal hemangioblastomas.MethodsThis retrospective study included 14 consecutive patients treated for spinal hemangioblastomas at our institute between January 2000 and June 2013. The mean follow-up period was 5 years. Magnetic resonance imaging of the complete neuraxis was performed in all cases, and preoperative embolization was performed in two cases.ResultsIn total, 14 patients underwent 18 surgeries, of which 15 were for spinal hemangioblastomas. Of all the patients, 86% had motor weakness and 79% presented with sensory disturbances. Preoperative McCormick functional grades were grade I in 7 (50%), grade II in 3 (21%), and grade III in 4 (29%) patients; 50% patients were diagnosed with VHL disease. All patients underwent complete resection of the tumor. Eight patients experienced deterioration in their neurological status in the immediate postoperative period; among them, five had gradual improvement. At 5-year follow-up, 11 (78.57%) patients showed good functional outcomes.ConclusionsMicrosurgical excision of spinal hemangioblastomas can cause postoperative morbidity, mainly in the form of neurological deterioration. Almost half of our patients had deterioration in the McCormick grade in the immediate postoperative period. However, a complete microsurgical excision can result in good long-term functional outcomes, as most of the immediate postoperative neurological deterioration in our patients was reversible. There was no difference in the long-term functional outcomes between sporadic and VHL-associated spinal hemangioblastomas.

Surgical Treatment of Intramedullary Hemangioblastomas: Current State of Problem (Review)

“…The Table shows the largest series of surgeries for HAB resections. In most cases, it was a total tumor resection: in >90% patients (see, e.g., [2,26,[35][36][37][38][39][40][41][42]). Some series have an increased number of subtotal resections -15.0-37.5% [18,[43][44][45].…”

Spinal Haemangioblastomas: A Report of Two Cases

“…Common locations in the CNS include the cerebellum (44%-72%), spinal cord (13%-50%), brainstem (10%-20%), supratentorial region (<1%), and lumbosacral nerve roots (<1%) (5). Spinal cord hemangioblastomas are relatively rare, accounting 1,6 for only 2% to 6% of all spinal cord tumors. In spite of their benign nature, they can result in clinically devastating morbidities to the affected patients due to severe neurological deficits resulting from tumour 1,3,4,7 mass, perilesional oedema, cyst or syrinx.…”

Spinal Haemangioblastomas in Nigerians: A Report of Two Cases

“…Haemangioblastomas are benign vascular tumours of the central nervous system, CNS (1,2,3,4). They may occur sporadically (75%) or as a component of Von Hippel-Lindau (VHL) disease (25%) (1).…”

“…Haemangioblastomas are benign vascular tumours of the central nervous system, CNS (1,2,3,4). They may occur sporadically (75%) or as a component of Von Hippel-Lindau (VHL) disease (25%) (1). Common locations in the CNS include the cerebellum (44%-72%), spinal cord (13%-50%), brainstem (10%-20%), supratentorial region (<1%), and lumbosacral nerve roots (<1%) (5).…”

“…Common locations in the CNS include the cerebellum (44%-72%), spinal cord (13%-50%), brainstem (10%-20%), supratentorial region (<1%), and lumbosacral nerve roots (<1%) (5). Spinal cord haemangioblastomas are relatively rare, accounting for only 2% to 6% of all spinal cord tumors (1,6). In spite of their benign nature, they can result in clinically devastating morbidities to the affected patients due to severe neurological deficits resulting from tumour mass, perilesional oedema, cyst or syrinx (1,3,4,7).…”