Gliomatosis Cerebri Masquerades as a Granulomatous Process (P4.225)

Abstract

Background: Gliomatosis cerebri is a rare primary brain tumor characterized by diffuse neoplastic infiltration generally presenting in the 3rd or 4th decades of life. We present a case of gliomatosis mimicking a granulomatous process. Case: A 38-year-old woman with 6-8 weeks of nonspecific back pain and headache was brought to the hospital after being found unresponsive. Lumbar puncture revealed >1000 RBCs, 45 WBCs, and total protein >1000. MRI brain showed multiple areas of abnormal T2 prolongation and diffuse nodular leptomeningeal enhancement. She had an extensive workup for autoimmune (Anti-Ro/La, ANA, anti-dsDNA, anti-cardiolipin antibodies, RNP, RF), granulomatous (ANCA, ACE), infectious (encephalitis panel, VZV, HIV, Borrelia, Brucella, HTLV, Syphilis, Coccidiomycosis, Entamoeba, Mycobacterium, Cryptococcus, Rickettsia, Toxoplasma), demyelinating (oligoclonal bands, anti-NMO, MBP), toxicological and paraneoplastic (including anti-NMDA) disorders. Three CSF cytology and flow cytometry evaluations were unremarkable, as was CT chest/abdomen/pelvis. Her evaluation was notable for low levels of complement, positive serum JC virus, high CSF albumin, and low CD4 count (225). She was treated empirically with high-dose prednisone, ampicillin, ceftriaxone, and acyclovir, improved to baseline, was discharged home after 15 days, and resumed normal activities (e.g. laundry, cooking), albeit with increased fatigability. Unfortunately, she re-presented less than one week later with decreased oral intake, vomiting, and seizures. CT head revealed acute hydrocephalus. Repeat MRI brain revealed interval worsening of the nodular enhancement “consistent with granulomatous meningitis” and stable intraparenchymal T2 abnormality “suggestive of encephalitis”. MRI spine showed diffuse thoracolumbar nodular thickening and leptomeningeal enhancement. She underwent dural and optico-carotid lesion biopsies. The optico-carotid lesion revealed high-grade astrocytic tumor; she was diagnosed with gliomatosis cerebri. Her family elected to pursue a palliative course. She passed away less than 8 weeks after initial presentation. Conclusions: It is important to consider neoplastic etiologies in the differential of granulomatous processes, even when preliminary neoplastic evaluation is unremarkable.

Disclosure: Dr. LaBuzetta has nothing to disclose. Dr. Rama has nothing to disclose. Dr. Lemkuil has nothing to disclose. Dr. Karanjia has nothing to disclose.