The purpose of this paper was to define the histologic distrito the endemic (African) and sporadic forms of Burkitt lymbution, clinical features, and treatment response of childhood phoma, which differ in their clinical presentation, regional non-Hodgkin lymphoma (NHL) in northeastern Brazil. We incidence, association with Epstein-Barr virus, and biologic Patients and methodsThere was a striking predominance of the small noncleaved cell (Burkitt) subtype, which occurred in 92 of the 98 children and adolescents diagnosed with NHL. Subsequent analyses Between January 1980 and October 1987, a total of 98 chilfocused on these patients. The majority (n = 84) had advanced dren were diagnosed with non-Hodgkin lymphoma at the parental refusal/abandonment of therapy (10%). Epstein-BarrDiagnostic imaging studies were performed as indicated. virus (EBV) was detected in tumor cells from eight of the 11The pathologic diagnosis was established by histologic Of the 98 patients evaluated during the study period, 92 had small noncleaved cell histology. There were only five cases of lymphoblastic lymphoma and one of large cell histology. Having confirmed the predominance of Burkitt lymIntroduction phoma among the pediatric patients with NHL, we then focused on these 92 cases. Records were reviewed to evaluate The non-Hodgkin lymphomas of childhood are predominantly clinical and biological features, type of treatment, and outhigh-grade extranodal tumors that include the lymphoblastic, come. When sufficient paraffin-embedded tumor tissue was large cell, and small noncleaved cell subtypes. 1 The relative available, in situ RNA/RNA cytohybridization using plasmids frequency of these subtypes varies with geographic location.containing EBV-encoded small nuclear RNA was performed In the United States, the distribution is almost equal, 2 whereas at St Jude Children's Research Hospital to detect the presence small noncleaved cell tumors (Burkitt lymphoma) account for of Epstein-Barr virus DNA. 6 In these cases, the histologic diagthe majority of childhood NHLs in equatorial Africa. 3,4 In nosis was also reviewed and confirmed by a pediatric hematonortheastern Brazil -an area in which we have established a pathologist at St Jude (CWB). cooperative clinical program -anecdotal evidence suggests an increased overall incidence of lymphoma. However, to our knowledge, there have been no reports focusing on pediatric Hodgkin or non-Hodgkin lymphomas in this region. TreatmentWe noted an apparent predominance of the small noncleaved cell histology among children treated for NHL at a There were two distinct treatment periods. From 1980 to major pediatric cancer center in Recife, Brazil. In the present 1983, all consenting patients were treated with induction study, we evaluated the accuracy of this clinical impression chemotherapy similar to that used in the LSA 2 L 2 regimen and assessed long-term treatment outcome. We also sought to described by Wollner et al, 7 followed by maintenance therapy determine the relationship of the small noncleaved...