Von Hippel-Lindau Disease Manifestating as Recurrent Cerebellar Hemangioblastoma: A Case Report
Journal of Korean Neurosurgical Society
;
: 571-577, 1997.
Article
in Korean
| WPRIM
| ID: wpr-146804
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.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Pancreas
/
Arm
/
Prognosis
/
Brain Neoplasms
/
Infratentorial Neoplasms
/
Genes, Tumor Suppressor
/
Hemangioblastoma
/
Cystadenoma
/
Diagnosis
/
Von Hippel-Lindau Disease
Type of study:
Diagnostic study
/
Prognostic study
Limits:
Adult
/
Female
/
Humans
Language:
Korean
Journal:
Journal of Korean Neurosurgical Society
Year:
1997
Type:
Article
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