Primary spheno-orbital angiosarcoma: Case report and review of the literature

OBJECTIVE: Angiosarcoma is a rare neoplasm that uncommonly involves the cra nium. We report the second case in the literature of a primary right spheno -orbital malignant angiosarcoma. CLINICAL PRESENTATION: This 43-year-old man experienced a 3-month history o f rapid growing temporal mass. The results of his neurological examination were normal. Neuroimaging revealed an intensively enhanced right spheno-orb ital lesion, with destruction of the greater wing of the sphenoid bone and extension into the orbit, the infratemporal fossa, and the temporal fossa w ith infiltration of the dura mater but without parenchymal abnormality. INTERVENTION: The tumor was first biopsied and then totally removed, with e xenteration of the right eye. The histological features were typical of ang iosarcoma with immunohistochemical evidence of Factor VIII-related antigen produced by tumor cells. Neither radiotherapy nor chemotherapy was performe d. The patient remained well during 16 months of follow-up, without evidenc e of recurrence on magnetic resonance images. CONCLUSION: We emphasize the benefit of wide surgical resection without sys tematic complementary treatment (radiotherapy and/or chemotherapy) in a cas e of primary angiosarcoma of the cranium. The literature is reviewed.