抄録
A solitary hemangioblastoma of the optic nerve was found in a 36-year-old male with a distinct family history of intracranial hemangioblastoma. The patient was admitted with complaints of visual loss and exophthalmos of the right eye. X-rays showed enlargement of the right optic canal. Right carotid angiography revealed a hypervascular tumor in the orbital apex, supplied by the ophthalmic artery. Computed tomography disclosed a pear-shaped, isodense mass with moderate contrast enhancement in the orbital apex. The right optic nerve was enlarged along its entire course and was involved with the apical mass. Surgery via the right frontal extradural approach disclosed a solid, vascular tumor involving the optic nerve at the apex of the orbit. Histological examination showed the tumor to be a characteristic hemangioblastoma. Over 90% of intracranial hemangioblastomas are located in the posterior fossa. Supratentorial hemangioblastomas, especially those arising in the optic nerve, are extremely rare. In addition, optic nerve hemangioblastomas are frequently familial and are associated with infratentorial hemangioblastomas, angiomatosis retinae, and cysts of the abdominal viscera.
