Marginal Zone Lymphoma Causing Superior Sagittal Sinus Stenosis and Intracranial Hypertension

Dural Lymphomas (DL) are a rare type of primary CNS lymphoma, with typical Pathology consistent with marginal zone B cell lymphoma (MZL)2. The most common presenting symptoms of DL are seizures (41%), headaches (33%) and cranial nerve palsies (30%)1. We present a case of dural lymphoma causing intracranial hypertension due to critical stenosis of the superior sagittal sinus.

Poster 55 Marginal Zone Lymphoma Causing Superior Sagittal Sinus Stenosis and Intracranial Hypertension Mhafrin Basta1, Daniel Kornberg2, Anika Tandon2, Marc Dinkin2, Cristiano Oliveira2 D Y Patil University School of Medicine, Navi Mumbai, India, Mumbai, India, 2Department of NeuroOphthalmology, Weill Cornell Medical College, New York, New York, USA 1 Introduction: Dural Lymphomas (DL) are a rare type of primary CNS lymphoma, with typical pathology consistent with marginal zone B cell lymphoma (MZL)2. The most common presenting symptoms of DL are seizures (41%), headaches (33%) and cranial nerve palsies (30%)1. We present a case of dural lymphoma causing intracranial hypertension due to critical stenosis of the superior sagittal sinus. Description of Case(s): A 32-year-old man who presented for evaluation of incidental bilateral optic disc edema, allegedly for over one year, reporting transient vision obscuration in the left eye and rare headaches. On examination there was bilateral papilledema Frisén Grade 2 and 4, right and left eye, respectively, with normal afferent and efferent visual function. The contrast-enhanced MRI brain/orbits and MRV head revealed dural based enhancement along the left frontal and parietal convexity extending to the posterior falx cerebri with severe critical stenosis of the superior sagittal sinus. Subsequent spinal tap was unrevealing for malignant cells or signs of inflammation/infection but showed expected elevated opening pressure at 32cm H2O. Craniotomy for biopsy provided the diagnosis of MZL. The patient had history of MZL on cervical lymph node diagnosed and resected two years prior. He was prescribed acetazolamide and completed 14 sessions of radiation therapy over a period of one month. He continued with follow-up neuro-ophthalmic examinations and MRI/MRV head, with progressive resolution of the papilledema and radiological findings. Conclusions, including unique features of the case(s): The present case illustrates the indolent nature of DL1 with essentially asymptomatic bilateral papilledema for one year before diagnosis. Cerebral venous sinus stenosis by DL should be suspected in patients with prior history of systemic MZL presenting with bilateral papilledema. References: 1. Macarena I. de la Fuente, Aya Haggiagi, Adrienne Moul, Robert J. Young, Charif Sidani,et.al ,Marginal zone dural lymphoma: the Memorial Sloan Kettering Cancer Center and University of Miami experiences, 58:4, 882-888, DOI: 10.1080/10428194.2016.1218006,2016 2. Bayraktar S, Stefanovic A, Montague N, Davis J, Murray T, et.al, Central nervous system manifestations of marginal zone B-cell lymphoma, Ann Hematol ,Oct;89(10):1003-9, 2010 3. Fabio M. Iwamoto, MD; Lisa M. DeAngelis, MD; and Lauren E. Abrey, MD, Primary dural lymphomas: A clinicopathologic study of treatment and outcome in eight patients, NEUROLOGY ;66:1763- 1765, 2006 4. Wajeeha Razaq, Anupama Goel, Ali Amin, Michael L. Grossbard, Primary Central Nervous System Mucosa-Associated Lymphoid Tissue Lymphoma: Case Report and Literature Review, Clinical Lymphoma & Myeloma, Vol. 9, No. 3, E5-E9, 2009 5. Joon Young Choi, Ji Hwan Chung, Young Jun Park, Geun Yong Jung, Tae Wook Yoon, et.al ,Extranodal Marginal Zone B-Cell Lymphoma of Mucosa-Associated Tissue Type Involving the Dura. Cancer Res Treat;48(2):859-863,2016. Keywords: Tumors, High intracranial pressure/headache, Optic neuropathy Financial Disclosures: The authors had no disclosures. Grant Support: None. 110 | North American Neuro-Ophthalmology Society