ABSTRACT
Introduction 1201 Epidemiology 1202 Clinical presentations 1202 Initial workup and staging and initiation of treatment 1204 Treatment 1205 General considerations 1205
Treatment strategies and their evolution 1206 General guidelines and controversies 1210 Conclusion 1214 Summary 1214 Key points 1214 References 1214
Burkitt lymphoma was first described, as indicated by its name, by Dennis Burkitt in 1959, as a rapidly growing facial tumor occurring in black African children, especially males. This tumor seemed to occur preferentially in the subtropical belt and to be related to Epstein-Barr virus (EBV), particles of which were found in the tumor nuclei. Later, the same histologic aspects were found in ‘reticulosarcomas’ occurring in North American and European children in other sites, especially in the abdomen. Thus they were described the ‘endemic’ Burkitt lymphoma as described by Burkitt and related to EBV and the ‘nonendemic’ Burkitt lymphoma occurring in other parts of the world and usually not related to EBV. These two entities present the same histocytologic aspects but have some different clinical and biological features. An important phase in the understanding of the biology of the disease was the description of nonrandom chromosomal translocations involving the protooncogene MYC on chromosome 8q24 and one of the heavy μ or light κ or λ chain immunoglobulin constant region genes on chromosome 14q32 (the most frequent), 2p12, or 22q11, respectively.
