Burkitt Lymphoma-A Guide to Biological Features, Diagnosis and Differential Diagnosis

Burkitt lymphoma (BL) is an aggressive mature B-cell lymphoma that represents one of the most studied human malignancies. Initially described in equatorial Africa by the Irish surgeon Denis P. Burkitt, African (endemic) Burkitt lymphoma was the first human neoplasm shown to be associated with a virus, the Epstein-Barr virus (EBV), and also the first human neoplasm shown to harbor a recurrent chromosomal aberration, the t(8;14) (q24;q32) translocation that led to the identification of the central role of the MYC gene in tumorigenesis. In this review, we provide a brief historical introduction, followed by a presentation of important aspects of epidemiology, pathogenesis, and of diagnostic features including morphology, cytogenetics and molecular findings. We also provide a comprehensive overview of the findings convincingly demonstrating that subtyping of BL into EBV-positive and EBV-negative better describes the biological heterogeneity of this lymphoma entity than the historical subtyping into endemic, sporadic, and immunodeficiency-associated. As the distinction of BL from other B-cell lymphomas is important for providing optimal oncological care, we also discuss the differential diagnosis and how this lymphoma can be distinguished from other aggressive B-cell lymphomas.