Primary Neurolymphomatosis in the Cavernous Sinus and the Brainstem. A Case Report and Review of Literature

Abstract

Primary neurolymphomatosis (NL) is a rare condition that refers to lymphomas primarily presenting in cranial or peripheral nerves. Similar to primary central nervous system lymphoma (PCNSL), almost all cases of neurolymphomatosis are histologically classified as high-grade diffuse B cell lymphoma. Primary involvement of cranial nerves is extremely rare and manifests with signs and symptoms of mass effect. We present the case of a 67-year-old right-handed male who presented to hospital with progressive horizontal diplopia and left facial numbness in the V1 and V2 distributions. The patient subsequently developed ataxia, dizziness, and nausea. An open biopsy was performed which confirmed the diagnosis of diffuse B cell lymphoma. The patient experienced rapid clinical improvement after the administration of chemotherapy. A comprehensive search of literature using the MEDLINE and EMBASE database yielded 9 previous cases of primary neurolymphomatosis in the cranial nerves and these are reviewed here. However, to date, this is the first reported case involving the trigeminal nerve in the cavernous sinus while concurrently causing mass effect on the lower cranial nerves.