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Neuroradiology-neuropathology conferenceFelipe Espinoza, MDUniversity of North Carolina at Chapel HillJune 2013Case 158 yo female with decreased vision OD

Diagnosed with bilateral granulomatous pan-uveitis

1/2013 presented with dizziness, vision changes & ear discharge

3Case 1 ContinuationHad a brain biopsy & started treatment

Patient develop left eye blindness 2 days after brain surgery

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 involvementMayo Rochester5

Primary CNS lymphoma1ry 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.

6080% with PVRL without CNS involvement subsequently develop PCNSL.

Median survival after CNS involvement is reported to be 1218 months.1ry vitreoretinal lymphomaKatoch D, et al. BMJ Case Rep 2013. doi:10.1136/bcr-2013-009354Case 212 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

Empirically treated for viral encephalitis & sent home.

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

Outside CT: negative

Case 2 ContinuationFollow up MRI: increased FLAIR & enhancement is subarachnoid tissues

Spine MRI: leptomeningeal enhancement

Negative MRA

Negative 4 vessel DSA

MR 2.20.201311

MR 2.21.201212Case 2 ContinuationResponded to steroids

Unclear diagnosis

Readmitted 6 weeks after onset of symptoms

CSF: negative

Case 2Lumbosacral hemilaminotomy, dural biopsy, intradural exploration & filum terminale biopsy

Diagnosis: Diffuse leptomeningeal oligodendroglioma15

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Suprasellar cistern covered with white tumor.17

Frontal horns of lateral ventricles filled with tumor.18Diffuse 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):275-8.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):1122-6.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):627-41.

Disseminated leptomeningeal oligodendroglioma19Case 374 yo female with speech difficulty & RUE weakness

Relapsing & remitting course

Diagnosis: gliomatosis cerebri

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Outside CT21Case 449 yo male bilateral hemianopsiahistory of esthesioneuroblastomas/p 2 cycles of chemo therapychemotherapy resistant disease

Transferred for fever & possible meningitisDiagnosis: atypical pituitary adenoma

4/2013 pre surgery23Case 69 yo male had a seizure while playing video games

No prior pertinent history

Went to brain biopsy: dyembryoplastic neuroepthelial tumor WHO grade IBenign cortical pointing towards the ventricles, demarcated wedge shapedscalloped25Case 6 Diagnosis: DNET