Clinicopathological Study of Sporadic Burkitt Lymphoma in Children

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Background:Non-Hodgkin lymphoma is the fourth most common malignant tumors in children,Burkitt lymphoma (BL) accounts for 30-50% of all pediatric lymphomas.The aim of this study was to investigate the clinicopathologic features,immunophenotype,Epstein-Barr virus (EBV) infection and c-myc gene rearrangement of sporadic BL in children.Methods:Ninety-two cases of pediatric BL were retrospectively analyzed for clinical features,immunohistochemistry,EBV-encoded RNA (EBER) status by in situ hybridization and c-myc gene rearrangement by fluorescence in situ hybridization.Results:In the 92 cases,male is predominant in sex distribution (M:F =3.38:1).The average age at diagnosis was 4.97 years.Polypoid BL showed a lower clinical stage (P =0.002),and advanced clinical stage and low serum albumin level at diagnosis were associated with poor outcome (P =0.024 and 0.053,respectively).The positive expression of CD10,B-cell lymphoma-6,MUM1 and EBER were 95.7% (88 cases),92.4% (85 cases),22.8% (21 cases),41.3% (38 cases),respectively.The expression of MUM1 were not associated with EBV infection status (P =1.000).c-myc gene rearrangement was detected in 94.6% (87/92).Clinical treatment information for 54 cases was collected,21 patients died of tumor after surgery alone,33 patients received surgery and chemotherapy,and of which six patients died shortly afterwords (MUM 1 positive expression in 3 cases,P =0.076).Conclusions:The anatomical location,growth patt and serum albumin level of BL were associated with biological behavior.MUM1 may be a potential adverse prognostic marker,and not associated with EBV infection status.
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