A Rare Case of Giant Solid Hemangioblastoma Accompanied with Hemophilia Type A

Abstract

The surgical removal of giant solid hemangioblastoma involves a high risk of perioperative bleeding and requires attentive hemostasis. Here, we present a case of a giant solid hemangioblastoma accompanied with hemophilia which was previously undiagnosed. A 35-year-old man without any past medical history was admitted with diplopia and ocular motility disorder. computed tomography (CT) and magnetic resonance imaging (MRI) revealed obstructive hydrocephalus and a solid giant tumor of more than 4.0 cm in diameter in the right cerebellopontine angle (CPA). Hemangioblastoma was suspected on cerebral angiography. After ventriculoperitoneal shunt for obstructive hydrocephalus, oozing from the skin incision continued for several days. Hemophilia type A was diagnosed based on the result of laboratory blood coagulability examination. Supplemental administration of factor VIII and coil embolization of the feeding arteries of the lesion on the CPA were performed, and the tumor was subtotally resected without hemorrhagic complications. The histopathological diagnosis was hemangioblastoma. We report this case to emphasize the importance not to overlook previously undiagnosed coagulopathy before surgical excision of hemangioblastoma. And, with appropriate perioperative management for coagulopathy, surgical treatment involving a high risk of perioperative bleeding can be safely undertaken.