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Journal of Korean Neurosurgical Society 1997;26(4): 563-570.
Three Cases of Cerebellar Hemangioblastoma in a von Hippel-Lindau Family: Case Report.
Jung Hoon Han, Chul Ku Jung, Ji Ho Yang, Chang Myung Choi, Il Woo Lee, Joon Ki Kang
Department of Neurosurgery, Catholic University Medical College, Seoul, Korea.
ABSTRACT
Hemangioblastoma is one of the benign tumors in the central nervous system. It is often associated with von Hippel-Lindau disease(VHL disease), a well known autosomal dominant hereditary tumor syndrome. We have experienced three cases of hemangioblastoma associated with von Hippel-Lindau disease in a same family, mother(case 1, 58 years old), son(case 2,19 years old, dead) and daughter(case 3, 17 years old). All of them had cerebellar hemangioblastomas. Renal cyst was associated in the case of mother, but not in the son or daughter. Hemangioblastoma associated with von Hippel-Lindau disease frequently occurres in multiple regions such as cerebellum, spinal cord, medulla oblongata. Also it is frequently presented with renal cell carcinoma, pheochromocytoma, cyst in pancreas, kidney and liver, and epididymal cystadenoma. The age of onset of von Hippel-Lindau disease in the second generation was younger than that of the first generation in this family.
Key Words: Hemangioblastoma; von Hippel-Lindau disease; Autosomal dominant hereditary tumor syndrome
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