NEURORADIOLOGY- NEUROPATHOLOGY CONFERENCE FELIPE ESPINOZA, MD University of North Carolina at Chapel Hill June 2013

Case 1 58 yo female with decreased vision OD 58 yo female with decreased vision OD Diagnosed with bilateral granulomatous pan- uveitis Diagnosed with bilateral granulomatous pan- uveitis 1/2013 presented with dizziness, vision changes & ear discharge 1/2013 presented with dizziness, vision changes & ear discharge

Case 1 Continuation Had a brain biopsy & started treatment Had a brain biopsy & started treatment Patient develop left eye blindness 2 days after brain surgery Patient develop left eye blindness 2 days after brain surgery Vitreous fluid cytometry: Large B cell lymphoma Vitreous fluid cytometry: Large B cell lymphoma

3 of 55 patients during an 11- year period had primary vitreous involvement without systemic symptoms 5% of patients with systemic lymphoma had vitreous involvement

Primary CNS lymphoma

1ry vitreoretinal lymphoma (PVRL) is a subset of primary CNS lymphoma (PCNSL). PVRL presents in the eye with or without simultaneous CNS involvement. An extranodal non-Hodgkin, diffuse large B-cell lymphoma. Median age of onset is late 50s and 60s with a female preponderance. 25% of patients with PCNSL without eye involvement subsequently develop PVRL. 60–80% with PVRL without CNS involvement subsequently develop PCNSL. Median survival after CNS involvement is reported to be 12–18 months. 1ry vitreoretinal lymphoma Katoch D, et al. BMJ Case Rep doi: /bcr

Case 2 12 yo female, transferred from OSH with 3 week history of HA, numbness of hands & feet & slurred speech 12 yo female, transferred from OSH with 3 week history of HA, numbness of hands & feet & slurred speech CSF -protein of 256, opening pressure of 55 CSF -protein of 256, opening pressure of 55 Empirically treated for viral encephalitis & sent home. Empirically treated for viral encephalitis & sent home.

Case 2 No improvement & presented to ER again after severe HA with slurred speech & inability to recognize her mother, right arm twitching No improvement & presented to ER again after severe HA with slurred speech & inability to recognize her mother, right arm twitching Outside CT: negative Outside CT: negative

Case 2 Continuation Follow up MRI: increased FLAIR & enhancement is subarachnoid tissues Follow up MRI: increased FLAIR & enhancement is subarachnoid tissues Spine MRI: leptomeningeal enhancement Spine MRI: leptomeningeal enhancement Negative MRA Negative MRA Negative 4 vessel DSA Negative 4 vessel DSA

Case 2 Continuation Responded to steroids Responded to steroids Unclear diagnosis Unclear diagnosis Readmitted 6 weeks after onset of symptoms Readmitted 6 weeks after onset of symptoms CSF: negative CSF: negative

Case 2 Lumbosacral hemilaminotomy, dural biopsy, intradural exploration & filum terminale biopsy Lumbosacral hemilaminotomy, dural biopsy, intradural exploration & filum terminale biopsy Diagnosis: Diffuse leptomeningeal oligodendroglioma Diagnosis: Diffuse leptomeningeal oligodendroglioma

Suprasellar cistern covered with white tumor.

Frontal horns of lateral ventricles filled with tumor.

Diffuse leptomeningeal gliomatosis is a rare condition in which glioma grows primarily within the subarachnoid space rather than within the brain parenchyma. While the majority of these diffuse leptomeningeal gliomas are astrocytic, several cases of diffuse leptomeningeal oligodendroglioma have been reported, including primary cases where no intraparenchymal lesions were identified. Many of the cases of diffuse leptomeningeal oligodendroglioma have occurred in children. Diagnosis can be difficult, since CSF generally shows elevated protein levels without neoplastic cells, and only fibrosis may be evident in the biopsy. Histology often demonstrates round, oligodendroglioma-like cells with perinuclear halos. While these tumors are often low-grade, anaplastic progression can occur and is associated with a shorter survival time. In a series of 9 patients who died from this disease, survival ranged from 3 months to 21 yrs. Mathews MS, Par LS, Kuo JV, Kim RC. Primary leptomeningeal oligodendrogliomatosis. J Neurooncol. 2009; 94(2): Ozkul A, Meteoglu I, Tataroglu C, Akyol A. Primary diffuse leptomeningeal oligodendrogliomatosis causing sudden death. J Neurooncol. 2007; 81(1):75-9. Armao DM, Stone J, Castillo M, Mitchell KM, Bouldin TW, Suzuki K. Diffuse leptomeningeal oligodendrogliomatosis: radiologic/pathologic correlation. AJNR Am J Neuroradiol. 2000; 21(6): Rodriguez FJ, Perry A, Rosenblum MK, Krawitz S, Cohen KJ, Lin D, Mosier S, Lin MT, Eberhart CG, Burger PC. Disseminated oligodendroglial-like leptomeningeal tumor of childhood: a distinctive clinicopathologic entity. Acta Neuropathol. 2012;124(5): Disseminated leptomeningeal oligodendroglioma

Case 3 74 yo female with speech difficulty & RUE weakness 74 yo female with speech difficulty & RUE weakness Relapsing & remitting course Relapsing & remitting course Diagnosis: gliomatosis cerebri Diagnosis: gliomatosis cerebri

Case 4 49 yo male bilateral hemianopsia 49 yo male bilateral hemianopsia history of esthesioneuroblastoma history of esthesioneuroblastoma s/p 2 cycles of chemo therapy s/p 2 cycles of chemo therapy “chemotherapy resistant” disease “chemotherapy resistant” disease Transferred for fever & possible meningitis Transferred for fever & possible meningitis Diagnosis: atypical pituitary adenoma Diagnosis: atypical pituitary adenoma

Case 6 9 yo male had a seizure while playing video games 9 yo male had a seizure while playing video games No prior pertinent history No prior pertinent history

Case 6 Diagnosis: DNET Diagnosis: DNET <1% of 1ry brain tumors <1% of 1ry brain tumors 1% of neuroepithelial tumors in patients older than 20 years of age 1% of neuroepithelial tumors in patients older than 20 years of age Reported in 5-80% of epilepsy specimens Reported in 5-80% of epilepsy specimens

Dysembryoplastic neuroepithelial tumor (DNET)