Burkitt lymphoma in children and adolescents: a single-center analysis over a 30-year period

Introduction and objectives: Burkitt lymphoma (BL) is a fast-growing and highly aggressive type of non-Hodgkin lymphoma. Nevertheless, 80-90% of patients on risk-adapted chemotherapy survive. The aim of this study was to characterize a pediatric population with BL in a center of reference, determine the 30-year incidence and calculate event-free and overall survival. Methods: We performed a retrospective and observational analysis of all patients diagnosed with BL between January 1993 and December 2022. Demographic and clinical data was collected. Patients were staged according to the St. Jude classification system and treated with different protocols according to risk stratification. A descriptive analysis, Portuguese central region incidence calculation and survival analysis were performed. Results: A total of 48 patients were included, 85% of which were male, with a mean age of 7.8 ± 3.7 years. Most cases (95.8%) were sporadic. The mean incidence rate was 0.34 cases per 100,000 person-years. Most (50%) patients were St. Jude stage III. A total of 41% were admitted to the pediatric intensive care unit (PICU), mostly due to tumor lysis syndrome (78.9%). The mortality rate was 6.3%. The mean follow-up was 87.8 ± 59.0 months with a 3-year event-free survival rate of 88.9% and overall survival rate of 93.2%. Subgroup analysis showed no differences between chemotherapy protocols. Higher uric acid was independently associated with PICU admission. Discussion: Our results regarding incidence and mortality are in line with previously-published literature. Notably, the last patient who died in our center was diagnosed 18 years ago. In conclusion, our results contribute to a better understanding of the epidemiology, clinical course and outcomes of BL in Portugal’s pediatric population.