Intracranial venous sinus thrombosis as unusual presentation of Bing-Neel syndrome: Case illustration

A 42-year-old woman presented with progressive blurred vision, vertigo, and headache. MRI showed bilateral intraocular protrusion of the optic nerve head and right transverse sinus thrombosis . Low-molecular-weight heparin was administered without symptom relief. Additional MRI showed left frontoparietal arachnoid space obliteration by pathological tissue, and infiltration of the sagittal and right transverse sinuses. After Gd administration, we observed diffuse enhancement of the dura in the left cerebral hemisphere and subdural and epicranial soft-tissue infiltration . A biopsy of the extra-axial tissue was performed. Histological examination showed a neoplasm composed of large cells with ovoid nuclei associated with non-neoplastic lymphocytes. Frequent mitotic figures were observed in the large cells. The large neoplastic cells were positive for CD20, bcl-2, bcl-6, and MUM1 and negative for CD10 and CD30. Accordingly, a diffuse infiltrative form of Bing-Neel syndrome was diagnosed. The patient was treated with 3 cycles of rituximab, dexamethasone, methotrexate HD, and cytarabine. Osmotic and steroid therapies were undertaken.