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

Waldenstrom macroglobulinemia, a lymphoplasmacytic lymphoma, accounts for 2% of monoclonal gammopathies.2,4,5 Neurological complications occur in 25% of these patients5 with peripheral nerve infiltration or serum hyperviscosity syndrome.1,3,4 Direct malignant infiltration of the brain is rare and is referred to as Bing-Neel syndrome.1,3,5 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 (Fig. 1). 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 (Fig. 2). A biopsy of the extra-axial tissue was performed. Histological examination showed a neoplasm composed of large cells with ovoid nuclei associated with nonneoplastic 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 (Fig. 3). 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. However, a few days after the diagnosis she died of respiratory failure before initiation of brain radiotherapy. To our knowledge, this is the first report of diffuse neoplastic infiltration of the principal intracranial venous sinuses in Bing-Neel syndrome. The absence of specific symptoms makes diagnosis challenging. Clinical and neuroradiological evaluations can anticipate the diagnosis and allow early treatment. Therapeutic modalities, such as chemotherapy and radiotherapy alone or in combination, have been proposed for BingNeel syndrome treatment. However, the number of successfully treated cases is insufficient to suggest any statistical conclusion about the superiority of one treatment modality over another.