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Washington University Experience | VASCULAR | Vasculitis - PACNS | 12A0 Case 12 History
Case 12 History ---- The patient is a 58-year-old woman who presented with altered mental status, cortical blindness and left extremity weakness and multiple episodes of confusion since Nov to early Dec 2020. It happened at the time when she had been using amphetamine. Initially considered as drug-induced encephalopathy versus demyelinating condition, the patient was treated with methylprednisolone on 1/1. MRI shows patchy areas of confluent T2/FLAIR hyperintensity throughout both cerebral hemispheres predominantly affecting the deep white matter with superimposed foci of diffusion restriction and susceptibility artifact as well as evolving areas of enhancement. The differential diagnosis included vasculitidies as well as toxic/metabolic processes. Operative procedure: Right parietal open biopsy. ---- H&E stained sections show cerebral cortex, scant subcortical white matter and attached leptomeninges with robust involvement of multiple blood vessels by a malignant neoplasm with large tumor cells harboring scant cytoplasm, vesicular nuclei, single to multiple prominent nucleoli, and conspicuous mitotic activity. In some areas chronic non-neoplastic inflammation rich in lymphocytes is seen in the leptomeninges, including rare foci of lymphocytic vasculitis with occasional acute/subacute microinfarcts in the adjacent cortex. Immunohistochemical studies show the intravascular tumor cells are uniformly positive for CD20, negative for CD3, ALK-1 and CAM5.2. CD3, however, does highlight the mature T-cells infiltrating into the leptomeninges, Virchow-Robin spaces and sparsely throughout the brain parenchyma. The collective morphological and immunohistochemical features are consistent with an intravascular large B-cell lymphoma. There is the potential for diagnostic confusion with small biopsies.
