Treatment Strategy of Multiple Hemangioblastomas

OBJECTIVE: Hemangioblastomas are highly vascular and benign neoplasm of the central nervous system(CNS). They can often be found as multiple lesions, as is commonly observed in von Hippel-Lindau(VHL) disease. The aim of this study is to determine the proper management for multiple hemangioblastomas. METHODS: Since 1990, 78cases of hemangioblastoma have been encountered. Among these, 9cases were multiple hemangioblastomas that were treated with surgical resection with or without radiosurgery. The medical, radiological, surgical and histological records were reviewed retrospectively and analyzed statistically. RESULTS: Nine patients presented with multiple hemangioblastomas and were diagnosed as VHL disease. The mean follow-up duration was 75.7months (6.6~159.2months) after the first surgical treatment. Three patients were treated with surgical resection alone and six patients were treated by both surgical resection and radiosurgery. Twenty-one surgical procedures (13 surgical resections and 8 radiosurgery) were performed. One patient required ventriculoperitoneal shunt and a posterior fossa decompressive craniectomy because of post-radiation brain swelling. Another patient refused additional treatment for the newly developed lesions after the successful treatment of initial lesions. The other patient who presented with numerous lesions in the whole brain and spine underwent cranio-spinal irradiation. Remaining patients showed good results. CONCLUSION: The surgical outcomes for the patients with a single lesion of the CNS hemangioblastoma are favorable. However, the treatment of multiple hemangioblastoma is more difficult, and should be treated by surgical resection and radiosurgery with careful consideration.

Two Cases of Multiple Hemangioblastomas in a Von Hippel-Lindau Family

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.

Multiple Hemangioblastomas in Cerebellum, Medulla Oblongata and Spinal Cord

Hemangioblastomas comprise 1 to 2% of all intracranial neoplasm, and 8 to 12% of tumors within the posterior fossa. They are composed of admixtures of three different cell types; endothelial cells, pericytes and stromal cells. Although most hemangioblastomas arise sporadically, they are associated with von Hippel-Lindau disease in about 20% cases. We have experienced a case of multiple hemangioblastomas occuiing in the cerebellum, medulla oblongata and cervical spinal cord simultaneously in a 55-year-old male. He had complained of headache, dizziness, generalized weakness and gait disturbance for 2 weeks. The patient had neither specific family history nor increased hematocrit. MRI showed a nonenhancing cystic lesion with an enhancing mural nodule in the right cerebellar hemisphere and two separate enhancing nodules in the medulla oblongata and dorsal cervical spinal cord at the 5-6th. Grossly, the excised mass of the cerebellum, 2.5 x 2 x 1.8cm, was solid to partly cystic, and that of spinal cord, lcm in diameter, was mostly solid. Microscopically, the tumor was composed of thin-walled blood vessels in variable size and interspersed stromal cells. The stromal cells revealed dimorphic cytoplasm that were either homogeneous and eosinophilic, or clear and vacuolated. Immunohistochemically, the endothelial cells reacted positively for glial fibrfllaty acidic protein(GFAP) and vimentin. The stromal cells reacted diffusely positively for vimentin, focally positively for GFAP and S-100 protein near the periphery of the tumor, focally positivel for neuro specipic enolase(NSE), and negatively for lysozyme, desmin and chromogranin. Ultrastructurally, the stromal cells contained numerous microfilaments and lipid droplets.

Multiple Hemangioblastomas in Cerebellum, Medulla Oblongata and Spinal Cord

Hemangioblastomas comprise 1 to 2% of all intracranial neoplasm, and 8 to 12% of tumors within the posterior fossa. They are composed of admixtures of three different cell types; endothelial cells, pericytes and stromal cells. Although most hemangioblastomas arise sporadically, they are associated with von Hippel-Lindau disease in about 20% cases. We have experienced a case of multiple hemangioblastomas occuiing in the cerebellum, medulla oblongata and cervical spinal cord simultaneously in a 55-year-old male. He had complained of headache, dizziness, generalized weakness and gait disturbance for 2 weeks. The patient had neither specific family history nor increased hematocrit. MRI showed a nonenhancing cystic lesion with an enhancing mural nodule in the right cerebellar hemisphere and two separate enhancing nodules in the medulla oblongata and dorsal cervical spinal cord at the 5-6th. Grossly, the excised mass of the cerebellum, 2.5 x 2 x 1.8cm, was solid to partly cystic, and that of spinal cord, lcm in diameter, was mostly solid. Microscopically, the tumor was composed of thin-walled blood vessels in variable size and interspersed stromal cells. The stromal cells revealed dimorphic cytoplasm that were either homogeneous and eosinophilic, or clear and vacuolated. Immunohistochemically, the endothelial cells reacted positively for glial fibrfllaty acidic protein(GFAP) and vimentin. The stromal cells reacted diffusely positively for vimentin, focally positively for GFAP and S-100 protein near the periphery of the tumor, focally positivel for neuro specipic enolase(NSE), and negatively for lysozyme, desmin and chromogranin. Ultrastructurally, the stromal cells contained numerous microfilaments and lipid droplets.