Primary central nervous system lymphoma: an update

The purpose of the present review is to present an overview of the recent findings in diagnostic procedures, treatment outcomes and biological advances in primary central nervous system lymphoma (PCNSL).

Recent imaging techniques are helpful in the diagnosis of atypical presentations of PCNSL and are likely to represent useful tools for patient follow-up. Knowledge of the biology of PCNSL is still fragmentary, but an increasing amount of data support the biological specificity of this disease with the identification of specific molecular alterations in PCNSL and specific interactions of lymphoma cells with the CNS microenvironment. The origin of the malignant cell is still unknown. Improvement of the therapeutic results along with numerous phase II studies clearly highlight a few important issues that need to be unequivocally answered and stress the need for the development of multicentric comparative studies. Attempts to decrease treatment-related toxicity on the CNS without impacting the disease control are ongoing.

The standard of care for PCNSL has definitively switched toward a curative objective. Considerable cooperative efforts are being made and will hopefully result in both a better understanding of the disease and significant therapeutic outcomes.