Key result
Paediatric patients with sporadic Burkitt lymphoma had significantly higher 1-year overall survival compared to adults (80% vs 15%; P=0.001), despite similar phenotypic and genotypic features.
Why the study?
Do sporadic paediatric and adult Burkitt lymphomas differ in phenotypic/genotypic features and survival outcomes?
Observational (n=31)
Do sporadic paediatric and adult Burkitt lymphomas differ in phenotypic/genotypic features and survival outcomes?
Absolute Event Rate: 80% vs 15%
p-value: p=0.001
Sporadic paediatric and adult Burkitt lymphomas share similar phenotypic and genotypic features, but adults have significantly poorer survival, potentially due to incorrect diagnosis and inappropriate treatment.
Adults with sporadic Burkitt lymphoma show markedly inferior survival despite similar features; leaves open whether diagnostic accuracy or treatment intensity explains the gap.
AIMS: To characterize the clinicopathological features of sporadic Burkitt lymphoma (BL). METHODS AND RESULTS: A retrospective study of 17 paediatric and 14 adult BLs with history and histopathology review, immunohistochemistry, Epstein-Barr virus (EBV) in situ hybridization (EBER) and fluorescence in situ hybridization. There was no statistically significant difference in gender, frequency of central nervous system (CNS) involvement and leukaemic change at presentation, or frequency of CD10+/Bcl-2-/Bcl-6+ (88% versus 86%), Ki67 labelling index, EBER (24% versus 21%), or C-MYC translocation (100% versus 92%) between paediatric and adult tumours. Correct pretreatment diagnoses were made in 13/17 (76%) paediatric and in 9/14 (64%) adult tumours. Twenty-eight patients received chemotherapy including 13/16 (81%) paediatric and 3/12 (25%) adult patients with appropriate regimens; 16 (57%) received CNS prophylaxis. The 1- and 5-year overall survival (OS) rates for paediatric patients were 80% and 50%, respectively, whereas 1-year OS for adults was 15%. CONCLUSIONS: Sporadic paediatric and adult BLs were phenotypically and genotypically similar. The significant prognosticators were age (P = 0.001), with or without CNS prophylaxis (P = 0.004), and CNS involvement (P = 0.008) and leukaemic change (P = 0.019) in disease course. The poor outcome in adult patients might be related to incorrect diagnosis and inappropriate treatment.
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Chuang et al. (2008) conducted an observational in Sporadic Burkitt lymphoma (n=31). Paediatric age vs. Adult age was evaluated on 1-year overall survival (p=0.001). Paediatric patients with sporadic Burkitt lymphoma had significantly higher 1-year overall survival compared to adults (80% vs 15%; P=0.001), despite similar phenotypic and genotypic features.
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