sporadic burkitt lymphomas of children and adolescents in chinese a clinicopathological study of 43 cases零星的伯基特淋巴瘤的儿童和青少年在中国43例临床病理研究.pdfVIP

Bi et al. Diagnostic Pathology 2012, 7:72 /content/7/1/72 RESEARCH Open Access Sporadic Burkitt lymphomas of children and adolescents in Chinese: a clinicopathological study of 43 cases * Cheng-Feng Bi, Yuan Tang, Wen-Yan Zhang, Sha Zhao, Xiao-Qing Wang, Qun-Pei Yang, Gan-Di Li and Wei-ping Liu Abstract Background: To investigate the clinical and pathologic features as well as the MYC translocations of childhood Burkitt lymphoma (BL) from China. Methods: Fourty-three cases of childhood BL were retrospectively investigated in morphology, immunophenotype, genotype, treatments and survival analysis. Results: Clinically, 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. Conclusions: Several differences were showed in this group of BL, including a much high