PREDOMINANCE AND CHARACTERISTICS OF BURKITT-LYMPHOMA AMONG CHILDREN WITH NON-HODGKIN-LYMPHOMA IN NORTHEASTERN BRAZIL

The purpose of this paper was to define the histologic distribution, c linical features, and treatment response of childhood non-Hodgkin lymp homa (NHL) in northeastern Brazil. We reviewed medical records and his topathologic studies of 98 children treated for NHL from 1980 to 1987 at a major pediatric cancer center in Recife, Brazil. Treatment outcom e was evaluated in relation to tumor burden (stage and serum LDH) and type of therapy (LSA(2)L(2) vs other multiagent chemotherapy). There w as a striking predominance of the small noncleaved cell (Burkitt) subt ype, which occurred in 92 of the 98 children and adolescents diagnosed with NHL. Subsequent analyses focused on these patients. The majority (n = 84) had advanced (stage III/IV) disease at diagnosis. The abdome n was the most common site of disease (84 cases); jaw involvement was rare (three cases). Five-year event-free survival (excluding treatment refusals) was significantly better for patients with limited vs advan ced stage disease (75 +/- 14% vs 42 +/- 6%; P < 0.04). Elevated serum LDH (>500 U/I) was associated with a poorer outcome (P = 0.008). The t ype of chemotherapy did not affect EFS (P = 0.95). Only 39% of patient s are long-term survivors, reflecting the high rate of septic deaths ( 25% of patients) and parental refusal/abandonment of therapy (10%). Ep stein-Barr virus (EBV) was detected in tumor cells from eight of the 1 1 cases studied. In clinical presentation, these cases resemble sporad ic Burkitt lymphoma, yet in their apparent responsiveness to LSA(2)L(2 ) therapy and association with EBV, they do not. Childhood NHL in nort heastern Brazil is predominantly of the Burkitt subtype, and is associ ated with clinical features that appear to distinguish it from the end emic and sporadic forms of this tumor. These cases may represent a thi rd or intermediate subtype of Burkitt lymphoma.