Résumé
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.
Résumé
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.