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Spinal cord hemangioblastomas in von Hippel-Lindau disease
- Source :
- Spinal cord. 47(6)
- Publication Year :
- 2008
-
Abstract
- Retrospective data analysis. To clarify the clinical features and surgical management of spinal cord hemangioblastomas in patients with von Hippel–Lindau disease (VHL). Clinical VHL Research Group in Japan, Japan. 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. 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. 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.
- Subjects :
- Adult
Male
medicine.medical_specialty
von Hippel-Lindau Disease
Adolescent
Central nervous system
Severity of Illness Index
Central nervous system disease
Young Adult
Hemangioblastoma
Medicine
Humans
Syrinx (medicine)
Spinal Cord Neoplasms
Von Hippel–Lindau disease
Retrospective Studies
Neurologic Examination
business.industry
Vascular disease
General Medicine
Middle Aged
medicine.disease
Spinal cord
Surgery
medicine.anatomical_structure
Treatment Outcome
Neurology
Cerebellar hemangioblastoma
Female
Neurology (clinical)
business
Subjects
Details
- ISSN :
- 14765624
- Volume :
- 47
- Issue :
- 6
- Database :
- OpenAIRE
- Journal :
- Spinal cord
- Accession number :
- edsair.doi.dedup.....2189740505233a5514b0d160bcf0ecc3