|
|
|
Journal of Korean Neurosurgical Society 1997;26(4): 571-577. |
| Von Hippel-Lindau Disease Manifestating as Recurrent Cerebellar Hemangioblastoma: A Case Report. |
| Kwan Uk Yoo, Chul Ku Jung, Il Woo Lee, Chang Myong Choi, Ji Ho Yang, Chun Kun Park, Sang Won Lee, Joon Ki Kang |
| Department of Neurosurgery, Catholic University Medical College, Seoul, Korea. |
|
|
|
|
|
| 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. |
| Key Words:
Recurrent Hemangioblastoma; von Hippel-Lindau disease; Tumor suppressor gene; Cystadenoma; Omental cyst |
|
|
|
Full text via DOI 
Download Citation 
