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Journal of Korean Neurosurgical Society 1999;28(12): 1782-1788. |
| Two Cases of Multiple Hemangioblastomas in a Von Hippel-Lindau Family. |
| Seung Won Kwak, Sin Soo Jeun, Kwan Sung Lee, Byoung Cheol Son, Yong Kil Hong, Chun Keun Park, Moon Chan Kim, Joon Ki Kang |
| Department of Neurosurgery, Catholic University Medical College, Seoul, Korea. |
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| ABSTRACT |
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Hemangioblastoma is uncommon, accounting for 1 to 2.5% of all primary neoplasms of the central nervous system. Ten to 20% of hemangioblastomas occur as part of Von Hippel-Lindau disease(VHL). Multiple hemangioblastomas are seen only with VHL and these are seen in up to half of VHL-associated hemangioblastomas. We have treated two cases of multiple hemangioblastomas associated with VHL disease in a family, sister(case I, 48/female) and her brother(case II, 41/male).
Both patients had renal and pancreatic cysts in addition to CNS hemangioblastomas. Their hemangioblastomas were removed totally, and then their neurological symptoms had Improved.
With a review of the literature, the authors present a family of VHL-associated multiple hemangioblastomas. |
| Key Words:
Multiple hemangioblastomas; Von Hippel-Lindau disease.; Renal cyst; Pancreatic cyst |
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