Clinicopathological Study of Sporadic Burkitt Lymphoma in Children

Abstract

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 CDl0, B-cell lymphoma-6, MUMl 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 (MUM1 positive expression in 3 cases, P = 0.076).Conclusions:The anatomical location, growth pattern 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.

Survival predictors of Burkitt's lymphoma in children, adults and elderly in the United States during 2000–2013

“…For example, Huang et al . 49 found low albumin and MUM1 expression to be highly correlated with increased mortality in BL. Another study found BCL-6 expression to be associated with better survival.…”

“…Several genetic markers and laboratory parameters have been identified as important prognostic markers but were not available for our analyses. For example, Huang et al 49 found low albumin and MUM1 expression to be highly correlated with increased mortality in BL. Another study found BCL-6 expression to be associated with better survival.…”

Intraoral EBV-positive sporadic Burkitt lymphoma in an elderly patient with bilateral presentation

“…Although CD10-positivity can be seen in DLBCL NOS, the neoplastic cells are usually Bcl-2 positive and display a slightly lower Ki-67 proliferative index compared with BL that is close to 100% 7. Although the present case was not assessed for gene rearrangement and MYC immunohistochemical staining, there are cases in the literature without information about MYC gene rearrangement, and according to clinical, morphological and immunohistochemical characteristics were diagnosed as BL 20. It is well recognized that chromosomal translocations in the MYC oncogene are the biological hallmark of BL, but also may be present in other B-cell lymphomas; in this sense, when the morphological characteristics are compatible with BL, and the immunohistochemical findings show positivity for MYC and Bcl-2, it is recommended to perform molecular tests in the search for gene rearrangements to rule out DHL or THL 1 , 11 , 16.…”

Outcome and survival in children with Non-Hodgkin’s lymphoma

“…8,9 BL is the most common histological subtype as mentioned in literature and as manifested in this study and commonest presenting sign decribed in literature was abdominal mass as it is evident in this study as well. 10,11 NH-Lymphomas are rapidly growing tumors and patients present late with widespread disease, majority of patients (>90%) in this study also presented in advanced stage i.e. in stage, 3 or 4.…”