Burkitt lymphoma in Papua New Guinea--40 years on.

摘要

BACKGROUND: Burkitt lymphoma is common in tropical Africa and Papua New Guinea, where it has been reported to account for 16% of all childhood malignancies. AIM: This study aimed to compare the geographical distribution of recent cases and their anatomical site of presentation with findings from previous studies, and to determine survival using the current treatment protocol. METHODS: The study included all cases of Burkitt and Burkitt-like lymphoma in children up to 14 years of age diagnosed between January 1998 and December 2003. RESULTS: Thirty-six children were diagnosed with Burkitt lymphoma, accounting for 50% of all lymphomas and 13% of all childhood malignancies. The median age was 6 years (interquartile range 4-8 years) and the male:female ratio was 8:1. Facial structures were the most commonly affected sites, accounting for 21 (58%) cases, followed by spinal involvement in three. The majority (89%) of patients came from malaria-holo-endemic, coastal PNG and three were from the highland region. The national incidence was 1.7/100,000 but provincial rates varied, the highest of 13.4/100,000 being in Gulf province. Only two of the 16 patients who received chemotherapy were known to be in remission at 12 months. CONCLUSIONS: Burkitt tumour remains a common childhood malignancy in PNG. There is a need to improve diagnosis and reporting so that treatment can be started early. The most appropriate treatment regimen for use in PNG and other resource-poor countries remains to be determined.