Gliomatosis Cerebri: the Great Masquerader Abstract Gliomatosis

Gliomatosis Cerebri: The Great Masquerader Abstract Gliomatosis cerebri, an infiltrative aggressive glioma, manifests a unilateral hemianopic field defect OS which was confounded by Advanced Glaucoma. I. Case History 90 year old white male, new patient requests glaucoma medication from the VA. He wishes to continue glaucoma follow up with his private ophthalmologist. Denies ocular complaints, CVA, confusion, headaches, aphasia. • Ocular, medical history: Advance Glaucoma OS>OD, Ocular Migraine, S/P CEOU, ALT OU, History of Ocular Trauma • Medications: Timolol .5% gel QAM OU, AlphaganP BID OU, Travatan Z QHS OU II. Pertinent findings • Clinical: VA OD: 20/20 OS: 20/25+1 o Objective: EOM: Full without diplopia or pain OU, pursuits and saccades intact OU, Confrontation visual field: OD full to finger counting, OS superior temporal restriction to central 10-20 degrees, Pupils: PERRL, (-) APD. o Anterior Segment Examination: Unremarkable, IOP OD 10 OS 10 o Posterior Segment Examination: Nerve- OD Pale C/D: 0.85v x 0.80h with very thin superior and sup/tem rims; OS Pale C/D: 0.95v x 0.90h with notching inferior-temporally and very thin superior rims,. Otherwise unremarkable. o HVF 24-2: OD scattered focal paracentral VF loss in double arcuate pattern, OS dense temporal hemifield loss with nasal encroachment. • Radiology studies: MRI March 2017 in Diagnosis and Discussion. III. Differential diagnosis: • Primary: Gliomatosis Cerebri • Others: Visual field: Pituitary tumor- Bitemporal hemianopia, Stroke of Posterior cerebral artery- Homonymous hemianopia. MRI: Viral Encephalitis, Multiple Sclerosis, Neurosarcoidosis, Vasculitis. IV. Diagnosis and discussion • Gliomatosis Cerebri (GC) is a rare, infiltrative, aggressive glioma that stems from neoplastic glial cells while preserving the integrity of the surrounding tissue. • Diagnosed with 1. Brain MRI: Criteria: Three or more contiguous lobes with T2 hyperintense and FLAIR showing the degree of infiltration. 2. If the Brain MRI meets the criteria, biopsy is needed to confirm Gliomatosis Cerebri. Acquired GC tissue shows diffuse and irregular infiltration of neoplastic astrocytes, oligodendroglial and mixed phenotype with minimal to absent vascularity. Molecular profiling is an additional step to determining the prognostication and treatment options • Often misdiagnosed, thereby delaying management o Non-specific symptoms from the most common headaches to hemiplegia o Radiologic imaging mimics viral encephalitis to neuroscarodosis Visit #1 We obtained his ophthalmologist’s medical notes including one visual field from 2014. Patient has advanced glaucoma OS>OD, visual field OS has an inferior arcuate defect and a left temporal hemianopia. No MRI or CT was ordered by the ophthalmologist. Patient was to continue his drops. Visit #2 Patient requests for transfer care to VA. HVF 24-2 was repeated March 2017 and showed a repeatable temporal hemianopic field defect OS. Brain MRI was ordered MRI report: Liew 1

• Mass effect involving right frontal lobe and temporal lobe with effacement of the involved sulci. • Increased hyperintensity in T2 and in FLAIR in the right basal ganglia. • A midline shift from right into left hemisphere: • FLAIR hyperintensity of the corpus callosum, with mass effect cross into left frontal lobe • A subfalcine shift is in the right cingulate gyrus herniating toward the left. Patient was diagnosed with Gliomatosis Cerebri in March 2017 by the radiologist through MRI. He was referred to neurosurgery and oncology. He declined biopsy. Follow up MRI June 2017 was stable. HVF testing July 2017 stable OU. V. Treatment, management • No standardized treatment o Adult prognosis median <1 year without anti-tumor therapy. Survival with treatment is up to 38 months. Radiation and chemotherapy are controversial. Surgical resection is avoided due to diffuse nature of tumor. Palliative therapy is preferred. o Current studies:Developing a biodata bank to better understand nature and migration pattern of Gliomatosis Cerebri, thereby avoiding misdiagnosis, and improving treatment and management. • Patient’s assessment and plan o Advanced Glaucoma OS>OD: Continue glaucoma drops, counseling on driving, o Gliomatosis Cerebri: Continue f/u with oncology, neuro-surgeon, counseling on driving, palliative care. Educate risk for seizures. Bibliography 1. Surabhi, Ranjan, et al.” Gliomatosis cerebri: Current Understanding and Controversies.” Frontiers in Oncology 7.1645 (2017) Web 11 August 2017. 2. Greenfield, Jeffrey P., et al. "Gliomatosis cerebri: A consensus summary report from the First International Gliomatosis cerebri Group Meeting, March 26–27, 2015, Paris, France." Pediatric blood & cancer 63.12 (2016): 2072-2077. 3. Rudà, Roberta, Luca Bertero, and Marc Sanson. "Gliomatosis cerebri: a review." Current treatment options in neurology 16.2 (2014): 273. 4. Essig, M., et al. "Fluid-attenuated inversion-recovery MR imaging of gliomatosis cerebri." European radiology 11.2 (2001): 303-308. 5. Carroll, Kate T., et al. "Management and Survival Patterns of Patients with Gliomatosis Cerebri: A SEER-Based Analysis." World Neurosurgery 103 (2017): 186-193. 6. Taillibert, Sophie, et al. "Gliomatosis cerebri: a review of 296 cases from the ANOCEF database and the literature." Journal of neuro-oncology 76.2 (2006): 201-205. 7. Desclée, P., et al. "Gliomatosis cerebri, imaging findings of 12 cases." Journal of neuroradiology 37.3 (2010): 148-158. 8. Ramanathan, Ramnath Santosh, Konark Malhotra, and Thomas Scott. "Teaching NeuroImages: diffuse cerebral neurosarcoidosis mimicking gliomatosis cerebri." Neurology 81.7 (2013): e46-e46.

VI. Conclusion • Neuro-ophthamic testing, such as red cap desaturation, confrontations, formal visual fields, pupils and color vision, is very important to uncover hidden ocular and or neurologic conditions • When ocular testing does not match the clinical picture, then additional testing, such as MRI, is warranted to investigate for masquerading diagnoses

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