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Journal of Korean Neurosurgical Society > Volume 26(4); 1997 > Article
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.
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
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