Spinal cord hemangioblastomas in von hippel-lindau disease: management of asymptomatic and symptomatic tumors.
OBJECTIVE
Standard treatment of asymptomatic spinal cord hemangioblastoma in von Hippel-Lindau (VHL) disease has yet to be established. The purpose of this study was to propose guidelines for the treatment of asymptomatic spinal cord hemangioblastomas in VHL disease.


METHODS
VHL disease patients treated for spinal cord hemangioblastomas between 1999 and 2009 were included. All spinal cord hemangioblastomas were divided into three groups: Group 1, asymptomatic tumors at initial diagnosis followed with serial imaging studies; Group 2, asymptomatic tumors at initial diagnosis that were subsequently resected; and Group 3, symptomatic tumors at initial diagnosis, all of which were resected.


RESULTS
We identified 24 spinal cord hemangioblastomas in 12 patients. Groups 1, 2 and 3 comprised 13, 4 and 7 tumors, respectively. Group 1 exhibited a smaller tumor volume (257.1 mm³) and syrinx size (0.8 vertebral columns) than those of Group 2 (1304.5 mm³, 3.3 vertebral columns) and Group 3 (1787.4 mm³, 6.1 vertebral columns). No difference in tumor volume or syrinx size was observed between Groups 2 and 3. Five tumors in Group 1 were resected during follow-up because symptoms had developed or the tumor had significantly grown. Finally, among 17 asymptomatic tumors at the initial diagnosis, nine tumors were resected. Only one tumor of these nine tumors resulted in neurological deficits, while five of seven symptomatic tumors caused neurological deficits.


CONCLUSIONS
Selective resection of asymptomatic tumors before they cause neurological deficits might bring about better outcomes.
Department of Neurosurgery, Yonsei University College of Medicine, 50 Yonsei-ro, Seodaemun-gu, Seoul, Korea.
Tae Yup
TY
Kim
Do Heum
DH
Yoon
Hyun Chul
HC
Shin
Keung Nyun
KN
Kim
Seong
S
Yi
Jae Keun
JK
Oh
Yoon
Y
Ha
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D018325:Hemangioblastoma / Q000209:etiology / Q000473:pathology* / Q000601:surgery*; D006801:Humans; D008297:Male; D008875:Middle Aged; D016896:Treatment Outcome; D006623:von Hippel-Lindau Disease / Q000150:complications*