Introduction: Lumbosacral nerve root hemangioblastomas in von Hippel-Lindau disease (VHL) can underlie significant neurologic signs and symptoms. Despite their associated morbidity, their surgical management and the functional outcomes of resection are not well defined. To define the results of surgical management of lumbosacral hemangioblastomas, we analyzed functional outcomes after surgery for these lesions.
Methods: VHL patients that underwent surgical resection of lumbosacral hemangioblastomas at the National Institutes of Health and the University of Virginia were included. Clinical and radiologic follow-up was performed at 6- to 12-month intervals after surgery. Functional outcome was defined using the McCormick clinical grading scale.
Results: Fourteen patients (7 male, 7 female) underwent 18 operations for 21 lumbosacral hemangioblastomas. Most patients presented with multiple symptoms including pain (12 patients), numbness (9), urinary complaints (6), and weakness (2). Mean combined preoperative tumor volume was 2.6±3.6 cm3 (range 0.3 to 15.0 cm3). Four tumors at 3 operations were not resected due to a motor nerve root origin. Gross total resection was achieved in 13 (87% of operations when resection was attempted). All tumors originated from within the involved nerve fascicle. Median follow-up was 5.9 years. New neurologic symptoms were noted after 11 operations (61%) but most (7; 64%) resolved within 2 weeks of surgery. At 6 months follow-up, 15 patients were stable, 2 patients were improved, and 1 patient was worse. Thirteen patients (72%) noted improvement of their presenting symptoms.
Conclusions: VHL-associated lumbosacral hemangioblastomas can be associated with motor nerve roots and may be large and challenging to manage surgically. Despite these findings, most patients improve with surgery, which should remain the primary therapeutic modality for patients with symptomatic lesions.
Patient Care: An understanding of the outcomes of surgery for lumbosacral hemangioblastomas in VHL is critical to defining the role of surgery in management for these patients.
Learning Objectives: By the conclusion of this session, participants should be able to:
1) Understand the typical presentation of lumbosacral hemangioblastomas in VHL
2) Describe the risks and benefits of surgical resection
3) Understand the role of surgery in the management of VHL-associated lumbosacral hemangioblastomas