Von Hippel-Lindau Disease Manifestating as Recurrent Cerebellar Hemangioblastoma: A Case Report
Journal of Korean Neurosurgical Society
; : 571-577, 1997.
Article
en Ko
| WPRIM
| ID: wpr-146804
Biblioteca responsable:
WPRO
ABSTRACT
Cerebellar hemangioblastoma comprises 2% of all brain tumors and 7% 10% of all posterior fossa tumors. It can arise in isolation("sporadic cases") or as a major manifestation of von Hippel-Lindau(VHL) disease, a well known autosomal dominant inherited tumor syndrome. Only 5-30% of these tumors are due to VHL disease. However, cerebellar hemangioblastoma occurs in younger patients, is often multiple and recurrent, and has a poorer prognosis than sporadic cases. We present a case of a 26-year-old woman with a right cerebellar hemangioblastoma, which recurred from a left cerebellar hemangioblastoma resected four years previously. Further evaluation established the diagnosis of VHL disease by demonstrating a cystadenoma in the pancreas and an omental cyst. Recently, the von Hippel-Lindau disease gene has been identified as a tumor suppressor gene and has been mapped to the short arm of chromosome 3(3p 25-26). Its absence or a defect in its structure is responsible for predisposition to the disease.
Palabras clave
Texto completo:
1
Base de datos:
WPRIM
Asunto principal:
Páncreas
/
Brazo
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Pronóstico
/
Neoplasias Encefálicas
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Neoplasias Infratentoriales
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Genes Supresores de Tumor
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Hemangioblastoma
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Cistoadenoma
/
Diagnóstico
/
Enfermedad de von Hippel-Lindau
Tipo de estudio:
Diagnostic_studies
/
Prognostic_studies
Límite:
Adult
/
Female
/
Humans
Idioma:
Ko
Revista:
Journal of Korean Neurosurgical Society
Año:
1997
Tipo del documento:
Article