Cavernous Sinus Syndrome as the Presentation of Systemic Non-Hodgkin’s Lymphoma

Abstract

Background: Cavernous sinus (CS) involvement has rarely been reported in malignant lymphoma. CS syndrome is uncommon as an initial presentation of non-Hodgkin’s extra nodal lymphomas. Case report: A 57-year-old man presented with a one-month history of headache, ocular pain and diplopia. Neurological examination revealed incomplete palsy of the left III and right VI nerves, and sensory loss of the first division of the left trigeminal nerve. Initial Magnetic Resonance Imaging (MRI) suggested left CS thrombosis. Despite optimal anticoagulation therapy, he developed right oculomotor nerve palsy, with ptosis and mydriasis of the left eye and bilateral sensory loss of the first and second division of the trigeminal nerves. MRI demonstrated a homogenous tissue lesion occupying the CS with moderate gadolinium enhancement. A body scan showed hepatosplenomegaly with hepatic and splenic nodules. The patient underwent percutaneous transhepatic biopsy and the lesion was histologically diagnosed as non-Hodgkin’s lymphoma, diffuse large B-cell type. Tumor cells were positive for CD20, CD79a and Ki67. Following four cycles of intravenous and intrathecal chemotherapy, the right oculomotor nerve palsy was completely resolved. There was partial improvement of enhancing lesion noted on follow-up MRI. Conclusion: CS syndrome is a rare presentation of malignant non-Hodgkin lymphoma. The diagnosis rests largely on imaging and biopsy results. It is associated with poor prognosis and Aggressive combined modality treatment appears to improve survival.