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Clinical features of patients bearing central nervous system hemangioblastoma in von Hippel-Lindau disease.
MedLine Citation:
PMID:  23080552     Owner:  NLM     Status:  Publisher    
BACKGROUND: Central nervous system (CNS) hemangioblastoma (HB) is one of the most common manifestations in von Hippel-Lindau disease (VHL), but large-scale studies on clinical features of CNS HB in VHL are scarce. METHODS: On the basis of the results of a questionnaire, we collected data of VHL patients with CNS HB. RESULTS: The total number of CNS HBs in 111 VHL patients (male 59, female 52) was 264 with the following distributions: cerebellar, 65.4 %; brainstem, 9.9 %; spinal cord, 23.9 %; and pituitary, 1. 1 %. The follow-up period was 0.6 to 39.2 years, with the mean 12.5 years. Patients bearing brainstem or spinal cord HB also had another HB significantly more frequently than those bearing cerebellar HBs (P < 0.05). The mean onset age of CNS HB was 29.1 years, and that of patients bearing a single HB (mean 34.4 years) was significantly greater than that of multiple HBs (mean 25.7 years). Patients with multiple HBs under 40 years are more dominant than those with a single HB. The distribution rate of brainstem HB is significantly smaller in patients below 30 years than patients above 29 years. Although ECOG PS score increased along with number of operations, the onset age decreased with increasing number of operations. The mean ECOG PS score of patients below 20 years is significantly smaller than patients above 19 years. CONCLUSIONS: When the onset age of CNS HB is under 40 years, and CNS HB is located at the brainstem or spinal cord HB, the probability of multiple occurrence can be predicted. Since patients with an onset age under 20 years old preserve a high performance status, early detection of CNS HB would be important. In addition, since a multiple operations aggravate performance status, number of operations should be reduced.
Hiroshi Kanno; Jun-Ichi Kuratsu; Ryo Nishikawa; Kazuhiko Mishima; Atushi Natsume; Toshihiko Wakabayashi; Kiyohiro Houkin; Shunsuke Terasaka; Taro Shuin
Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-10-19
Journal Detail:
Title:  Acta neurochirurgica     Volume:  -     ISSN:  0942-0940     ISO Abbreviation:  Acta Neurochir (Wien)     Publication Date:  2012 Oct 
Date Detail:
Created Date:  2012-10-19     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0151000     Medline TA:  Acta Neurochir (Wien)     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Department of Neurosurgery, Yokohama City University School of Medicine, 3-9 Fukuura, Kanazawa-ku, Yokohama, 236-0004, Japan,
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