An autopsy case of primary central nervous system lymphoma with diffuse intracerebral lesion

Abstract A case of primary central nervous system lymphoma (PCNSL) who initially showed clinical pictures like encephalitis and diffuse lesions on MRI was reported, including postmortem pathologic examinations. A 68-year-old woman was seen in March, 1990 with a 1-month history of the progressive gait disturbance. She was very unstable and could barely stand by herself, though she did not show any focal neurologic deficits. She showed no evidence of systemic diseases. The cerebrospinal fluid analysis was normal. T2-weighted image of MRI demonstrated the diffuse symmetric hyperintense lesions mainly in the periventricular white matter. The progressive intellectual decline and the spasticity of four limbs developed as the diffuse lesions on MRI gradually extended. Despite the administration of corticosteroids under the presumptive diagnosis of PCNSL, she rapidly fell into the apallic syndrome within two months. Her EEG showed periodic synchronous discharges. Three months later, she suddenly developed signs of right uncal herniation. CT showed a large mass lesion in the right hemisphere. After the anti-edema therapy, signs of herniation regressed. The serial CT scans demonstrated a gradual decrease in the mass effect, while another multi-nodular lesions appeared and then disappeared one after another bilaterally. Eventually, the diffuse low densities in the cerebral white matter and the ventricular enlargement had remained. She died of bronchopneumonia eight month after the onset of symptoms. The clinical importance of the diagnosis of PCNSL which initially shows diffuse symmetric lesions without a mass is stressed. Postmortem examinations revealed PCNSL, diffuse, large cell type according to the Lymphoma Study Group classification. The lymphoma cells were proved to be B cell origin from the immunohistochemical study of frozen tissue.(ABSTRACT TRUNCATED AT 250 WORDS)