Astroblastoma presenting with intracerebral hemorrhage misdiagnosed as dural arteriovenous fistula: review of a rare entity.

摘要

BACKGROUND: Astroblastoma is one of the most unusual types of tumors whose histogenesis has been recently clarified. It occurs mainly among children and young adults. It is grossly well demarcated and shows histologically characteristic perivascular pseudorosettes with frequent vascular hyalinization. Immunohistochemically, the tumor cells show diffuse strong positivity for GFAP, S-100 protein, vimentin, as well as neuron-specific enolase and focal positivity for EMA. CASE DESCRIPTION: Our patient is a 33-year-old gentleman who presented with intraparenchymal hemorrhage in the left temporal lobe. A 4-vessel cerebral angiogram revealed a dural AVF in the left posterior temporal area. The lesion was resected, and pathology showed a GFAP-positive lesion with prominent astroblastoma rosettes. There was abundant hemosiderin consistent with a prior hemorrhage. Because of its high degree of proliferation, the presence of astroblastic pseudorosettes, prominent perivascular hyalinization, regional hyaline changes, and pushing borders with regard to the adjacent brain, the tumor was considered anaplastic. CONCLUSIONS: Astroblastoma is a rare pure pathologic entity--a distinct form of astrocytic gliomas. The diagnosis of astroblastoma is often difficult because of the astroblastic aspects that can be found in astrocytic tumors, in ependymomas, and in nonneuroepithelial tumors. The natural history seems to be between astrocytomas and glioblastomas. Total resection and radiation therapy seem to be the advisable treatments.