[Hemangioblastoma and von Hippel-Lindau disease].
Intracranial hemangioblastoma (Lindau's disease) is vascular-rich benign neoplasm, arise from vascular endothelium. They account for about 2-3% of all intracranial tumors. Its most common location is adult posterior fossa. Except for the cerebellum, they were sometimes found in the brainstem or spinal region. Common macroscopical findings is mural nodule with cyst in the cerebellum. Contrary, solid form is common in spinal and brainstem region. The lesions are readily identified by image diagnostic procedures. Intracranial hemangioblastoma associated with retinal hemangioblastoma is called "von Hippel-Lindau's disease", hereditary disease of autosomal dominant form with chromosome abnormalities of 3p25-26. In other particular type of "von Hippel-Lindau's disease", intracranial hemangioblastomas are associated with renal cell carcinoma, pancreatic cyst or pheochromocytoma sometimes without retinal hemangioblastoma. Surgical extripation is the best choice of treatment for this disease. However, brainstem or spinal hemangioblastomas are sometimes difficult to remove totally.
Matsumoto
K
K
Department of Neurological Surgery, School of Medicine, University of Tokushima.
Kannuki
S
S
D000328:Adult; D002528:Cerebellar Neoplasms / Q000235:genetics / Q000473:pathology; D018325:Hemangioblastoma / Q000235:genetics / Q000473:pathology; D006801:Humans; D006623:von Hippel-Lindau Disease / Q000235:genetics / Q000473:pathology