Malignant lymphoma in African children: three decades of discovery.

The recognition of Burkitt lymphoma (BL) as a clinical syndrome and a pathological entity in African children resulted from astute clinical observations (bedside epidemiology), the availability of cancer registry data and accurate pathological interpretation. Following the early studies in Africa, it soon became evident that this tumor occurred worldwide and the excess of cases in Africa was an incidence phenomenon associated with specific environmental factors. The sentinel discovery of the Epstein Barr virus (EBV) and its association with BL stimulated a wide variety of scientific investigations which have had an impact of virtually every discipline and biology. Epidemiological observations linked to modern laboratory techniques have provided etiological insights which implicate specific environmental factors and genetic events in the pathogenesis of BL and other immunoproliferative diseases. Early infection with EBV and holoendemic malaria are clearly of paramount importance in the development of endemic BL (eBL). These factors do not play a role in the majority of sporadic BL (sBL) cases, but immunosuppression and T-cell deregulation almost certainly are common denominators. The final or principle genetic event in both instances would appear to be the chromosome 8 translocation involving the c-myc oncogene and structural alteration. It is expected that the BL model will continue to be a useful one for identifying basic mechanisms in carcinogenesis which may be applicable as well to a variety of non-neoplastic diseases.