TY - JOUR
T1 - Spinal cord hemangioblastomas in von hippel-lindau disease
T2 - Management of asymptomatic and symptomatic tumors
AU - Kim, Tae Yup
AU - Yoon, Do Heum
AU - Shin, Hyun Chul
AU - Kim, Keung Nyun
AU - Yi, Seong
AU - Oh, Jae Keun
AU - Ha, Yoon
PY - 2012/11
Y1 - 2012/11
N2 - Purpose: 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. Materials and 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. Conclusion: Selective resection of asymptomatic tumors before they cause neurological deficits might bring about better outcomes.
AB - Purpose: 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. Materials and 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. Conclusion: Selective resection of asymptomatic tumors before they cause neurological deficits might bring about better outcomes.
UR - http://www.scopus.com/inward/record.url?scp=84868134950&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84868134950&partnerID=8YFLogxK
U2 - 10.3349/ymj.2012.53.6.1073
DO - 10.3349/ymj.2012.53.6.1073
M3 - Article
C2 - 23074104
AN - SCOPUS:84868134950
VL - 53
SP - 1073
EP - 1080
JO - Yonsei Medical Journal
JF - Yonsei Medical Journal
SN - 0513-5796
IS - 6
ER -