Hemangioblastoma of spinal nerve: a report of six cases.
OBJECTIVE
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.


METHODS
The clinical resentations 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.


CONCLUSIONS
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.
Division of Laboratory Medicine and Automic Pathology, Mayo Clinic, Rochester, MN, USA. plaines@me.com
Andrew
A
Mitchell
Bernd W
BW
Scheithauer
Robert E
RE
Wharen
Joel
J
Franck
Kris
K
Chan
D000368:Aged; D005260:Female; D018325:Hemangioblastoma / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D013127:Spinal Nerves / Q000378:metabolism / Q000473:pathology*