Spinal Cord Hemangioblastomas in von Hippel-Lindau Disease: Management of Asymptomatic and Symptomatic Tumors

Department of Neurosurgery, Yonsei University College of Medicine, 50 Yonsei-ro, Seodaemun-gu, Seoul 120-752, Korea. .
Yonsei medical journal (Impact Factor: 1.29). 11/2012; 53(6):1073-80. DOI: 10.3349/ymj.2012.53.6.1073
Source: PubMed


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.

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.

Selective resection of asymptomatic tumors before they cause neurological deficits might bring about better outcomes.

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Available from: Jae Keun Oh, Aug 27, 2014
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    ABSTRACT: The authors present a retrospective analysis of 14 patients treated for spinal cord hemangioblastoma (SCH). This study was conducted to evaluate and compare the difference of functional outcomes associated with the extent of surgical removal of the tumor. Eleven patients were male and three were female, with a mean age of 37.2 years (19-62 years). Pre- and postoperative magnetic resonance imaging was performed in all cases. Preoperative angiography was performed in 11 cases and preoperative embolization in four. The follow-up period ranged from 15 to 161 months (median, 47 months). Thirteen patients had intramedullary tumor, and the remaining one had an extradural lesion. All patients underwent surgical removal, and total removal (TR) was achieved in 10 cases. In four patients with preoperative embolization, intraoperative bleeding was minimal and TR was possible. In three of four patients without TR, their functional outcomes were aggravated postoperatively. At the last follow-up (15-161 months), eight patients were improved, three were stationary, and three deteriorated. All patients who showed improvements underwent TR. The statistical analysis showed that TR produced a significantly better outcome than incomplete removal (p = 0.015). TR resulted in a better outcome for SCH. Preoperative embolization could be effective in the reduction of intraoperative bleeding and facilitate TR with an improved surgical outcome.
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