Primary central nervous system lymphoma (PCNSL) is a type of central nervous system restricted non-Hodgkin lymphoma, whose histopathological diagnosis is majorly large B cell lymphoma.
They are extranodal, of the diffuse large B cell type that is confined to the brain, eyes, leptomeninges, or spinal cord, in the absence of systemic lymphoma.
Primary cerebral lymphoma represents 4%–7% of primary brain tumors, and its incidence has increased in the last 3 decades 1).
Bailey first described PCNSL as “perithelial sarcoma” of the CNS and Henry in 1974 recognized its lymphoid origin 2).
Generally, PCNSL of DLBCL type is a highly aggressive tumor and although its prognosis is poor, about one-third of younger patients can hope for cure of the disease 3).
It typically presents as multifocal T2 hyperintense lesions that both enhance after gadolinium administration and demonstrate mass effect. A case study in 1999 coined the term ‘lymphomatosis cerebri’ to describe an exceedingly rare variant of PCNSL characterised by diffuse parenchymal infiltration of lymphomatous cells.
Although exceedingly rare, primary central nervous system lymphoma can present as growing intracerebral hemorrhage due to repeated intratumoral hemorrhages. High expression of vascular endothelial growth factor and the mass effects of hemorrhage could be associated with the onset and growth of intracerebral hemorrhage. Early evaluation and meticulous observation are important to avoid progressive, life-threatening situations in such cases 4).
Only three completed randomised trials are available for primary CNS lymphoma: one phase 3 and two phase 2 trials 5) 6) 7).