儿童肠道息肉样淋巴瘤15例临床病理分析

目的 观察儿童肠道息肉样淋巴瘤(PL)的临床病理学特征.方法 收集江西省儿童医院诊治的15例儿童肠道PL的临床病理资料并进行随访,免疫组织化学法检测CD10、bcl-6、bcl-2、MUM-1和ERCC1表达,原位杂交检测EB病毒编码的RNA(EBER).结果 15例肠道PL中伯基特淋巴瘤(BL)13例,患者CD10、bcl-6、bcl-2、MUM-1和ERCC1阳性率分别为100.0%(13/13)、92.3%(12/13)、0、7.7%(1/13)和15.4%(2/13),EBER阳性7例(53.8%);弥漫大B细胞淋巴瘤(DLBCL)和介于BL和DLBCL之间的未分类B细胞淋巴瘤(BL/DLBCL)各1例.13例BL患儿临床分期:Ⅱ期11例(84.6%),Ⅲ期和Ⅳ期各1例(7.7%);DLBCL和BL/DLBCL患者临床分期均为Ⅱ期.术后化疗的14例PL患儿均无瘤生存,随访时间24~120个月,1例术后未化疗的Ⅳ期BL患儿于术后2个月死亡.结论 儿童肠道PL以BL多见,临床分期较低,术后积极化疗预后好.

Clinicopathological analysis of intestinal polypoid lymphoma in children: report of 15 cases

Objective To investigate the clinicopathologic features of intestinal polypoid lymphoma (PL) in children. Methods The clinicopathologic data of 15 cases of pediatric intestinal PL in Jiangxi Provincial Children 's Hospital were retrospectively analyzed. Immuohistochemistry was used to detect the expressions of CD10, bcl-6, bcl-2, MUMl and ERCC1, then EB virus (EBV)-encoded RNA (EBER) status was tested by using in situ hybridization. Results Amongst 15 cases studied, 13 cases were Burkitt lymphoma (BL), 1 case was diffuse large B-cell lymphoma (DLBCL) and 1 case was between DLBCL and BL (DLBCL/BL). The positive rate of CD10, bcl-6, bcl-2, MUMl, ERCC1 and EBER were 100.0%(13/13), 92.3%(12/13), 0, 7.7 % (1/13), 15.4 % (2/13), 53.8 % (7/13), respectively in 13 BL cases. The clinical stage of 13 patients with BL: 11 cases (84.6 %) Ⅱ stage, 1 case (7.7 %) Ⅲ stage, 1 case (7.7 %) Ⅳstage. The clinical stage of DLBCL and BL/DLBCL: Ⅱstage. 14 cases of PL had survival time without tumors, 24-120 months follow-up, and 1 BL patient inⅣstage without chemotherapy died after 2 months. Conclusion BL is a major subtype of intestinal PL in children, which shows a low clinical stage and a good prognosis.