Comparative Analysis of Spinal Hemangioblastomas in Sporadic Disease and Von Hippel-Lindau Syndrome
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概要
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The clinical differences were evaluated in spinal hemangioblastoma between patients with sporadic disease and patients with von Hippel-Lindau (VHL) syndrome. The distribution of hemangioblastomas in the central nervous system was investigated in 56 patients treated between 1988 and 2008 at the University of Tokyo Hospital. The characteristics of spinal hemangioblastomas were compared in 35 patients including 17 with sporadic disease and 18 with VHL syndrome treated between 1988 and 2008 at our hospital and three affiliated institutions. Spinal hemangioblastomas were much more prevalent in patients with VHL syndrome (88.2%) than in patients with sporadic disease (20.5%, p < 0.001). Spinal hemangioblastomas associated with VHL syndrome were diagnosed a decade earlier (p = 0.007) and were associated with less severe neurological symptoms than sporadic lesions (p = 0.004). Most patients with sporadic disease had a single lesion at the cervical or thoracic level, whereas patients with VHL syndrome had multiple lesions at all spinal levels (p = 0.04). Patients with sporadic disease exhibited significant improvement in postoperative neurological status (p = 0.02), whereas patients with VHL syndrome had no change in status (p = 1.00). Number of removed lesions (p = 0.03) and lower spinal cord lesions (p = 0.05) were associated with poor surgical outcome. Tumor recurrence was correlated with partial removal of lesions (p = 0.05). One third of patients with VHL syndrome developed new lesions every 2 years. The major finding of this study is that the incidence of spinal hemangioblastoma, distributed through all spinal levels, may be as high as 88% in patients with VHL syndrome, which is much greater than previously reported.
- 社団法人 日本脳神経外科学会の論文
- 2010-07-15
著者
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TAKAI Keisuke
Department of Neurosurgery, The University of Tokyo Hospital
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TANIGUCHI Makoto
Department of Neurosurgery, Tokyo Metropolitan Neurological Hospital
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Saito Nobuhito
Department Of Neurosurgery Faculty Of Medicine Tokyo University
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Usui Masaaki
Department Of Electronics Shibaura Institute Of Technology
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Taniguchi Makoto
Department Of Bio- And Geoscience Gradaute School Of Science Osaka City University
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Takahashi Hiroshi
Department Of Applied Aquabiology National Fisheries University
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Takai Keisuke
Department Of Neurosurgery The University Of Tokyo Hospital
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