Abstract
BackgroundTo investigate the clinical and pathologic features as well as the MYC translocations of childhood Burkitt lymphoma (BL) from China.MethodsFourty-three cases of childhood BL were retrospectively investigated in morphology, immunophenotype, genotype, treatments and survival analysis.ResultsClinically, there was a marked male predominance in sex distribution (M: F = 9.75:1); abdomen was the most frequent extranodal sites of involvement (46.5 %), followed by jaws and facial bones (16.3 %). Two third of the patients were in stageI ~ II. Morphologically, 69.76 % of the cases showed classical histologic features, while 30.24 % of them showed greater nuclear pleomorphism in size and shape. Five cases (11.6 %) were positive for EBER1/2. Thirty-one of the 40 cases (77.5 %) had the aberration of IGH/MYC translocation while 7 (17.5 %) had non-IGH/MYC translocation. Thirty patients (69.7 %) received operation and/or chemotherapy while 13 patients (30.3 %) received no treatment. Twenty-seven patients (62.8 %) died of the tumor, 16 alive, with the average survival time 4.9 and 48.7 months respectively. High IPI, advanced clinical stage, increased serum level of LDH and no chemotherapy received as well as tumor size ≥10 cm were related to the lower survival rates of the tumor.ConclusionsSeveral differences were showed in this group of BL, including a much higher ratio of male patients, more cases in stageII, clinically inconsistent treatment and a very poor outcome.Virtual slidesThe virtual slide(s) for this article can be found here http://www.diagnosticpathology.diagnomx.eu/vs/1552295877710135
Highlights
To investigate the clinical and pathologic features as well as the MYC translocations of childhood Burkitt lymphoma (BL) from China
Clinical manifestations From 1990 to 2006, children and adolescents BL accounted for 0.3 % (60/18960) of all malignant lymphomas, 0.8 % (60/7429) of all non-Hodgin lymphoma and 7.1 % (60/850) of all lymphomas of children and adolescents in our hospital
Comprehensive clinical examination data were collected in 34 patients, with 24 patients presented with low international prognostic index (IPI) scores and 10 patients with high IPI scores respectively
Summary
To investigate the clinical and pathologic features as well as the MYC translocations of childhood Burkitt lymphoma (BL) from China. BL is a highly aggressive non-Hodgkin lymphoma (NHL) and characterized by C-MYC translocation. Three clinical variants of BL are recognized, including endemic BL, sporadic BL and immunodeficiency-associated BL. Sporadic BL is seen throughout the world, mainly in children and adolescents. The incidence of BL is low, accounting for only 1 ~ 2 % of all lymphomas in western countries, it is one of the most common types of malignant tumors in children and young adults [1]. 43 cases of BL of children and adolescents from southwest China were retrospectively investigated for the clinical and pathological features
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