Nodal diffuse large B-cell lymphomas in children and adolescents: immunohistochemical expression patterns and c-MYC translocation in relation to clinical outcome.
Nodal diffuse large B-cell lymphomas in children and adolescents: immunohistochemical expression patterns and c-MYC translocation in relation to clinical outcome.
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DOI：
10.1097/pas.0b013e3181bb9a18
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发表时间：
2009-12
期刊：
影响因子：
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通讯作者：
Bacchi CE
中科院分区：
文献类型：
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作者：
Gualco G;Weiss LM;Harrington WJ Jr;Bacchi CE
弥漫性大B细胞淋巴瘤（DLBCL）是一种非常罕见的肿瘤，在儿科年龄组，因此有很少的研究，免疫表型或遗传学的这些情况下。我们研究了16例发生在10至18岁之间的淋巴结DLBCL患者。根据2008年世界卫生组织分类标准，应用免疫组织化学方法检测CD 10、BCL-6和MUM 1蛋白，将淋巴瘤分为生殖中心型和非生殖中心型。此外，通过免疫组化评价TCL 1、BCL-2表达和Ki-67增殖指数，通过FISH评价c-MYC和BCL-2易位。所有这些参数与临床特征和结局相关。我们的研究显示，中心母细胞形态和生发中心型DLBCL在这些年轻患者中更常见（63%），其中37%含有c-MYC易位。只有1例显示BCL-2易位，反映了具有DLBCL和伯基特淋巴瘤中间特征的双重打击病例。我们发现BCL-2表达的频率比以前报道的更高，在单变量或多变量分析中对疾病的结果没有直接影响。以前没有专门研究过儿童淋巴结DLBCL中TCL 1的表达;我们发现TCL 1表达的发生率为31%。在44%的病例中观察到MUM 1表达，这些阳性病例对临床结果显示出显著的负面影响。TCL 1与c-MYC的存在和高增殖指数直接且显著相关。老年中心和非老年中心亚型的总生存期和无病期差异有统计学意义。在37%的患者中发现了C-MYC易位，并对临床结果产生了有利的影响。我们的结论是，淋巴结儿童和青少年DLBCL主要是生发中心型，尽管BCL-2的频繁表达和c-MYC易位的存在下，一个普遍良好的结果。TCL 1表达似乎与良好的临床结果相关，而MUM 1表达预测不良的临床结果。
Diffuse large B-cell lymphoma (DLBCL) is a very infrequent neoplasm in the pediatric age group; therefore there are very few studies on the immunophenotype or genetics of these cases. We studied a series of 16 patients with nodal DLBCL occurring in patients between 10 and 18 years of age. The cases were classified according to the 2008 World Health Organization classification criteria, with application of immunohistochemistry for the detection of CD10, BCL-6 and MUM1 proteins to divide the lymphomas into germinal center and non-germinal center types. In addition, TCL1, BCL-2 expression, and the Ki-67 proliferation index were evaluated by immunohistochemistry, and c-MYC and BCL-2 translocations were evaluated by FISH. All these parameters were correlated with clinical features and outcome. Our study revealed that centroblastic morphology and the germinal center type of DLBCL are more prevalent in these young patients (63%), with 37% containing a c-MYC translocation. Only one case showed a BCL-2 translocation, reflecting a double-hit case with features intermediate between DLBCL and Burkitt lymphoma. We found a higher frequency of BCL-2 expression than previously reported, with no direct influence on the outcome of the disease in univariate or multivariate analysis. The expression of TCL1 has not been specifically studied in nodal pediatric DLBCL before; we found a 31% incidence of TCL1 expression. MUM1 expression was observed in 44% of the cases and these positive cases showed a significant negative impact on clinical outcome. TCL1 is directly and significantly associated with the presence of c-MYC and a high proliferative index. The germinal center and non-germinal center subtypes showed significant differences for both overall survival and disease-free interval. C-MYC translocation was found in 37% of patients, and had a favorable impact on clinical outcome. We conclude that nodal pediatric and adolescent DLBCL are mainly of the germinal center type, with a generally good outcome in spite of the frequent expression of BCL-2 and the presence of c-MYC translocation. TCL1 expression seems to be associated with a good clinical outcome, while MUM1 expression predicts a poor clinical outcome.