Clinicopathological features of intravascular diffuse large B-cell lymphoma in the central nervous system

Abstract

Objective To investigate the clinicopathologic features of intravascular diffuse large B-cell lymphoma (IVLBCL) in the central nervous system (CNS). Methods The clinical and radiological data of three cases of CNS-IVLBCL in Xuanwu Hospital, Capital Medical University from 2014 to 2017 were collected. And pathological and immunohistochemical features of these patients were retrospectively analyzed. The related literatures were also reviewed. Results All the three patients aged from 62 to 76 years, with duration of 4-8 months. Clinical manifestations of the three patients included dizziness, fatigue, seizures, etc. They showed intracranial multiple lesions in the frontal, temporal, parietal and corona radiata, etc. MRI demonstrated cerebral infarction or space-occupying lesions. Microscopic observation showed small vessel lumina filled with tumor cells in the white matter. Tumor cells had large, round nucleus, and prominent nucleoli. The chromatin of tumor cells was broadly granular, and the mitotic figures were visible. Tumor cells expressed B cell markers, such as CD20, CD79α, paired box protein 5 (PAX-5). CD34 staining demonstrated that tumor cells were located in the lumen of the blood vessels. Two patients died within half a year after diagnosis, and the other one was lost to follow-up. Conclusions The clinical symptoms and MRI demonstrations of CNS-IVLBCL are variable and the prognosis is extremely poor. Morphological observation and immunohistochemical phenotyping for biopsy specimens are helpful for early diagnosis and actively combining chemotherapy to prolong survival of patients. Key words: Central nervous system; Lymphoma, large B-cell, diffuse; Pathology, clinical; Immunohistochemistry