Primary central nervous system lymphoma in neurosurgery

Abstract

Primary central nervous system lymphoma (PCNSL) most commonly (>95%) constitutes a specific kind of diffuse large B-cell lymphoma, which shows expression of CD20, CD19 and CD79a antigens on its surface and belongs to non-germinal center B-cell-like type (non-GCB). This results from both its limited localization as well as immunophenotypic and molecular features. It frequently has an aggressive clinical course and its prognosis remains highly uncertain. PCNSL’s development in areas normally free from lymphoid tissue has not been adequately explained thus far. PCNSL is usually a solitary lesion (60–70%), with the majority (c.60%) occurring in supratentorial areas, and less frequently in telencephalic nuclei and periventricular areas, corpus callosum, infratentorial structures, spinal cord or orbital cavities. Lymphoid cells can occasionally create diffuse infiltration with no mass effect — a PCNSL variant known as lymphomatosis cerebri. Reported clinical symptoms depend on the localization of the tumor in central nervous system. The most common include: cognitive impairment, behavioral changes, focal neurological deficit and symptoms of increased intracranial pressure. A final diagnosis of PCNSL requires histopathological evaluation of tissue samples obtained usually during a stereotactic biopsy. Identifying lymphoid cells in cerebrospinal fluid may also be sufficient. Chemotherapy combined with radiotherapy is the standard treatment of PCNSL. For many years, surgical treatment has been controversial. This provides constant encouragement to explore effective treatment methods, with neurosurgical involvement waiting to be further defined.