University of North Carolina at Chapel Hill
June 2013
NEURORADIOLOGYNEUROPATHOLOGY
CONFERENCE
FELIPE ESPINOZA, MD
Case 1
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58 yo female with decreased vision OD
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Diagnosed with bilateral granulomatous panuveitis
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1/2013 presented with dizziness, vision
changes & ear discharge
Case 1 Continuation
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Had a brain biopsy & started treatment
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Patient develop left eye blindness 2 days after
brain surgery
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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
• 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.
Katoch D, et al. BMJ Case Rep 2013. doi:10.1136/bcr-2013-009354
Case 2
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12 yo female, transferred from OSH with 3
week history of HA, numbness of hands & feet
& slurred speech
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CSF -protein of 256, opening pressure of 55
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Empirically treated for viral encephalitis &
sent home.
Case 2
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No improvement & presented to ER again
after severe HA with slurred speech & inability
to recognize her mother, right arm twitching
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Outside CT: negative
Case 2 Continuation
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Follow up MRI: increased FLAIR &
enhancement is subarachnoid tissues
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Spine MRI: leptomeningeal enhancement
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Negative MRA

Negative 4 vessel DSA
Case 2 Continuation
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Responded to steroids
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Unclear diagnosis
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Readmitted 6 weeks after onset of symptoms
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CSF: negative
Case 2

Lumbosacral hemilaminotomy, dural biopsy,
intradural exploration & filum terminale
biopsy
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Diagnosis: Diffuse leptomeningeal
oligodendroglioma
Suprasellar cistern covered with white tumor.
Frontal horns of lateral ventricles filled with tumor.
Disseminated leptomeningeal oligodendroglioma
•
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):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.
Case 3
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74 yo female with speech difficulty & RUE
weakness
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Relapsing & remitting course
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Diagnosis: gliomatosis cerebri
Case 4
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49 yo male bilateral hemianopsia
history of esthesioneuroblastoma
s/p 2 cycles of chemo therapy
“chemotherapy resistant” disease
Transferred for fever & possible meningitis
Diagnosis: atypical pituitary adenoma
Case 6
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9 yo male had a seizure while playing video
games
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No prior pertinent history
Case 6
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Diagnosis: DNET
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<1% of 1ry brain tumors
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1% of neuroepithelial tumors in patients older
than 20 years of age
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Reported in 5-80% of epilepsy specimens
Dysembryoplastic neuroepithelial tumor (DNET)