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Early microsurgical treatment for spinal hemangioblastomas improves outcome in patients with von Hippel-Lindau disease.
MedLine Citation:
PMID:  22347675     Owner:  NLM     Status:  PubMed-not-MEDLINE    
Abstract/OtherAbstract:
BACKGROUND: Spinal hemangioblastomas (HB) are rare, histologically benign, highly vascularized tumors often associated with von Hippel-Lindau (VHL) disease. The aim of the current study is to demonstrate the benefit of early surgical resection of large spinal HBs in selected asymptomatic patients with VHL.
METHODS: Seventeen patients underwent microsurgical resection of 20 spinal HBs at the Department of Neurosurgery at Helsinki University Central Hospital (HUCH). Thirteen tumors were in the cervical spine, five in thoracic and one patient had two lumbar lesions. MRI tumor showed an associated syrinx in 16 patients (94%). Tumor volume ranged from 27 to 2730 mm(3). Out of 17 patients, 11 (65%) tested positive for VHL in mutation analysis. Five of these patients with tumors ranging from 55 to 720 mm(3) were treated prophylactically.
RESULTS: Complete tumor resection was performed in 16 patients (94%) who were followed up for a median of 57 months (range 2-165 months). No patient had neurological decline on long-term follow-up. Among the patients with VHL, five patients with preoperative sensorimotor deficits showed improvement of their symptoms but never regained full function. One patient who presented with tetraplegia remained the same. Otherwise, all five patients with prophylactic surgery remained neurologically intact.
CONCLUSION: Although documented growth on serial MRIs and the need for pathological diagnosis have been suggested as indications for surgery in otherwise asymptomatic patients, our series showed that a potentially larger group of asymptomatic patients with spinal HB associated with VHL would benefit from microsurgical resection. Long-term results of the surgical management of spinal HB are generally favorable. Our results suggest staging and early treatment for spinal HB larger than 55 mm(3), especially in patients with VHL. Small spinal HBs may be followed up.
Authors:
Ali Harati; Jarno Satopää; Lydia Mahler; Romain Billon-Grand; Ahmed Elsharkawy; Mika Niemelä; Juha Hernesniemi
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Publication Detail:
Type:  Journal Article     Date:  2012-01-21
Journal Detail:
Title:  Surgical neurology international     Volume:  3     ISSN:  2152-7806     ISO Abbreviation:  Surg Neurol Int     Publication Date:  2012  
Date Detail:
Created Date:  2012-02-20     Completed Date:  2012-10-02     Revised Date:  2013-05-29    
Medline Journal Info:
Nlm Unique ID:  101535836     Medline TA:  Surg Neurol Int     Country:  India    
Other Details:
Languages:  eng     Pagination:  6     Citation Subset:  -    
Affiliation:
Department of Neurosurgical, Helsinki University Central Hospital (HUCH), Helsinki, Finland.
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