PMC7705382_01
(a) Initial Brain MRI Axial T2 FLAIR images demonstrating a large infiltrative FLAIR hyperintensity centered within the left frontal lobe with heterogeneous, incomplete peripheral enhancement and extension along the corpus callosum into the right frontal lobe. There were no diffusion restricting correlations, (b) Repeat Brain MRI Axial T2 FLAIR 2 days after initial brain MRI demonstrating interval progression of the large infiltrative FLAIR hyperintensity within the left frontal lobe with heterogeneous, incomplete peripheral enhancement and extension along the corpus callosum into the right frontal lobe, and (c) Repeat Axial T2 FLAIR after left hemicraniectomy and brain biopsy. There is persistent FLAIR hyperintensity within the left frontal lobe with heterogeneous, incomplete peripheral enhancement and extension along the corpus callosum into the right frontal lobe. There are residual blood products within the biopsy cavity.
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MRI of the brain (Figure 1(a)) was performed and showed a left frontal lobe lesion with vasogenic edema and midline mass effect. FLAIR (Figure 1(b)) sequence showed progression of the infiltrative lesion centered in the left frontal lobe with ring enhancement and patchy uptake within the lesion when compared to the outside hospital MRI. Funduscopic exam showed papilledema. Repeat brain MRI post-operatively showed persistent FLAIR hyperintensity in the left frontal lobe with heterogeneous, incomplete peripheral enhancement and extension of the hyperintensity across the corpus callosum in to the right frontal lobe (Figure 1(c)).
Lesion frontal lobe histology: (a) H&E: perivenous foci of palor and demyelination, collections of inflammatory cells and macrophages, (b) LFB-PAS: patchy perivascular demyelination with numerous fragments of myelin debris in macrophages, (c) CD68 and CD163: Myelin pallor and inflammatory cells showing demyelination with relative axon preservation accompanied by macrophages and a microglial and mononuclear cell infiltrate, and (d) Neurofilament: relatively well preserved axons in areas of demyelination.
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Pathology of the left frontal subcortical white matter showed multiple foci of perivascular chronic inflammation with pallor and aggregates of macrophages which were consistent with a demyelinating disorder (Figure 2).
Liver histology: (a) H&E x60: the biopsy of the liver shows complete replacement of the parenchyma with loosely cohesive cords and singly dispersed cells with rounded to irregular nuclei, and moderate amount of eosinophilic cytoplasm are dispersed in a hyalinized stroma and (b) ERG x60: marker for vascular differentiation including benign and malignant vascular tumors neoplasms such as hemangioendothelioma.
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At that time, she underwent a liver biopsy demonstrating EHE (Figure 3) but the patient was lost to follow-up.