Insights into the biology of primary central nervous system lymphoma.
Primary central nervous system lymphoma (PCNSL) is a rare variant of non-Hodgkin lymphoma that is confined to the central nervous system. Biologic studies of PCNSL are challenging to conduct because the disease is rare and available tissue material is sparse. However, in recent years there has been progress in the understanding of PCNSL biology, largely as the result of multicenter studies using modern molecular techniques. Recent studies may improve insight into the pathogenesis of PCNSL and increase the chances of identifying prognostic factors and novel therapeutic targets. This review discusses recent advances in PCNSL biology, including immunologic and genetic risk factors, and focuses on the molecular alterations important in central nervous system lymphomagenesis.
Mrugala
Maciej M
MM
Stephen E and Catherine Pappas Center for Neuro-Oncology, Yawkey 9E, Massachusetts General Hospital Cancer Center, Boston, MA 02114, USA.
Rubenstein
James L
JL
Ponzoni
Maurilio
M
Batchelor
Tracy T
TT
D014408:Biomarkers, Tumor; D002471:Cell Transformation, Neoplastic; D016543:Central Nervous System Neoplasms / Q000453:epidemiology* / Q000378:metabolism* / Q000473:pathology; D020869:Gene Expression Profiling; D004854:Herpesvirus 4, Human / Q000378:metabolism; D006801:Humans; D007150:Immunohistochemistry / Q000379:methods; D016130:Immunophenotyping; D008228:Lymphoma, Non-Hodgkin / Q000453:epidemiology* / Q000378:metabolism* / Q000473:pathology; D008495:Medical Oncology / Q000379:methods; D008954:Models, Biological; D008957:Models, Genetic; D009154:Mutation; D011379:Prognosis; D012307:Risk Factors
D014408:Biomarkers, Tumor