Document Detail

Spinal cord hemangioblastomas in von Hippel-Lindau disease.
MedLine Citation:
PMID:  19030009     Owner:  NLM     Status:  MEDLINE    
STUDY DESIGN: Retrospective data analysis.
OBJECTIVE: To clarify the clinical features and surgical management of spinal cord hemangioblastomas in patients with von Hippel-Lindau disease (VHL).
SETTING: Clinical VHL Research Group in Japan, Japan.
METHODS: Forty-eight out of 66 patients with associated spinal cord hemangioblastoma among 142 VHL patients were retrospectively examined with respect to clinical features, accompanying lesions and outcome of surgical treatment.
RESULTS: Among these 48 patients, 46 of them (95.8%) also had a central nervous system (CNS) hemangioblastoma at another site: 42 (87.5%) with cerebellar hemangioblastoma and 11 (22.9%) with brain stem hemangioblastoma. Twenty-three patients (47.9%) had more than one spinal cord hemangioblastoma. The 48 patients with spinal cord hemangioblastomas collectively had a total of 74 tumors. The tumor was accompanied with a syrinx in 64 and without it in 10 patients. Forty of the 48 patients underwent surgical treatment for their spinal cord hemangioblastomas, and 7 of these 40 underwent surgical treatment twice. When functional changes in the patients after these 47 operations were examined by postoperative evaluation by McCormick's classification, 39 of these operations (83.0%) resulted in improvement/no change and 8 (17.0%) in aggravation of symptoms.
CONCLUSION: Von Hippel-Lindau disease patients bearing spinal cord hemangioblastomas mostly had a CNS hemangioblastoma at another site. These tumors can be removed in the majority of VHL patients without aggravation. In these patients, when the timing of treatment for spinal cord hemangioblastoma is determined, the probability of occurrence and treatment of other lesions should be considered.
H Kanno; I Yamamoto; R Nishikawa; M Matsutani; T Wakabayashi; J Yoshida; N Shitara; I Yamasaki; T Shuin;
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Publication Detail:
Type:  Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't     Date:  2008-11-25
Journal Detail:
Title:  Spinal cord     Volume:  47     ISSN:  1476-5624     ISO Abbreviation:  Spinal Cord     Publication Date:  2009 Jun 
Date Detail:
Created Date:  2009-06-08     Completed Date:  2009-09-01     Revised Date:  2011-06-06    
Medline Journal Info:
Nlm Unique ID:  9609749     Medline TA:  Spinal Cord     Country:  England    
Other Details:
Languages:  eng     Pagination:  447-52     Citation Subset:  IM    
Department of Neurosurgery, Yokohama City University School of Medicine, Yokohama, Japan.
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MeSH Terms
Hemangioblastoma / etiology*,  surgery*
Middle Aged
Neurologic Examination
Retrospective Studies
Severity of Illness Index
Spinal Cord Neoplasms / etiology*,  surgery*
Treatment Outcome
Young Adult
von Hippel-Lindau Disease / complications*
Yukihiro Ibayashi / ; Toshihiko Yamaki / ; Yoshihiro Numagami / ; Eiji Jokura / ; Yoshimasa Kayama / ; Yuji Yamada / ; Yoshiaki Shiokawa / ; Junko Yamashita / ; Mitsuhiro Hasegawa / ; Hisashi Hatano / ; Jun Shinoda / ; Noboru Sakai / ; Waro Taki / ; Satoshi Matsushima / ; Kenichi Murao / ; Toshio Matsubara / ; Jun A Takahashi / ; Kengo Matsumoto / ; Hiroyuki Nakajima / ; Masanori Hashimoto / ; Shigeo Matsumoto / ; Kiyoshi Ichigizaki / ; Ikuro Murase / ; Kengo Kashiwabara / ; Yuzo Yamakawa / ; Hiromichi Yamazaki / ; Satoshi Kubo / ; Koichi Tokuda / ; Seisho Abiko / ; Hiromichi Miyazaki / ; T Anda / ; S Shibata / ; Tsunehiko Miyamoto / ; Naosumi Okawa / ; Shigebumi Morimoto / ; Michio Inoue / ; Mitsuhiro Miyagami /

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