We report a case of optic nerve hemangioblastoma in a 29-year-old man without a family history of von Hippel-Lindau disease or the other organ symptoms. He had progressive loss of vision and proptosis with a dull pain in his right eye. Magnetic resonance image (MRI) showed a gadolinium (Gd) enhancing solid tumor involving the prechiasmal right optic nerve with intraorbital extension. The tumor contained many signal voids of vessels and grew like a dumbbell through the right optic canal. Digital subtraction angiography demonstrated that the hypervascular tumor was fed by the ophthalmic artery and the orbital ramus of the middle meningeal artery. We succeeded in total resection of this hypervascular tumor by orbito-zygomatic approach. This tumor grew unusually like a dumbbell because it occurred in the optic nerve at the part of the optic canal. Histological examination revealed the tumor to be a characteristic hemangioblastoma. The postoperative course was uneventful. Supratentorial hemangioblastomas, especially optic nerve hemangioblastoma are extremely rare. This paper reviews the previous published cases and discusses their findings.

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