Aims: Hemangioblastomas may arise sporadically or in the setting of Von Hippel-Lindau (VHL) disease. In either instance, it rarely occurs outside the central nervous system. By analysis of a large case series, we sought to further characterize the clinical, radiologic and pathologic features of hemangioblastomas involving nerve root. Materials and methods: The clinical presentations of 6 proximal nerve root hemangioblastomas (1 an aggressive tumor) were analyzed with emphasis on the neuroimaging, operative, and pathologic findings. The literature is fully reviewed and updated. Results: Nerve hemangioblastoma usually affects proximal spinal roots. Peripheral nerve is rarely involved. Both clinically and radiologically, the diagnosis is usually not suspected before surgery. Profuse bleeding at resection may be the first indication of the nature of the lesion. These tumors may arise both sporadically and in association with VHL disease. Conclusion: Given their rarity, nerve root hemangioblastomas are not generally considered in the preoperative differential diagnosis of proximal nerve root lesions. Given their propensity to bleed profusely at surgery and the potential association with VHL disease, knowledge of this entity is important.
- Peripheral nerve
- Von Hippel-Lindau disease
ASJC Scopus subject areas
- Pathology and Forensic Medicine
- Clinical Neurology