扁桃体套细胞淋巴瘤的临床病理特征分析及文献复习

The analysis of clinicopathological characteristics of tonsillar mantle cell lymphoma

目的总结分析扁桃体套细胞淋巴瘤的临床病理特征,探讨其诊断及鉴别诊断要点。方法选取2005年1月至2014年5月在首都医科大学附属北京同仁医院确诊为扁桃体套细胞淋巴瘤的患者4例,对其临床资料、病理资料及随访结果进行回顾性分析,所有标本均按常规制片,行HE染色、免疫组织化学染色及CCND1免疫荧光原位杂交(FISH)检测。结果患者男3例,女1例,年龄53～74岁,中位年龄58岁,其中原位套细胞淋巴瘤1例,母细胞型套细胞淋巴瘤1例,经典型套细胞淋巴瘤2例。免疫组织化学染色结果:异型淋巴细胞CD20(+)、CD79a(+)、Pax5(+)、Cyclin D1(+)、CD5(+)、CD21生发中心FDC网(+)、CD23生发中心FDC网(+)、CD3(-)、CD45RO(-)、BCL2(-)、BCL6(-)、CD10(-)、TdT(-)。FISH检测结果为IGH/CCND1基因断裂。例1患者于2014年3月初次发病,未治疗,至2017年11月未见转移及复发。例2患者于2013年11月初次发病,经2次化疗后,2014年2月复发,2014年6月死亡。例3、例4的2例患者均获得随访,其中例3于2012年2月初次发病,经8次化疗后,至2017年11月,未见转移及复发;例4于2012年5月首次确诊,未治疗,2015年8月因病死亡。结论扁桃体套细胞淋巴瘤较其他部位套细胞淋巴瘤预后好。在扁桃体套细胞淋巴瘤中,原位套细胞淋巴瘤预后较好,母细胞性套细胞淋巴瘤预后相对较差,生存期较短。免疫组化染色Cyclin D1及免疫荧光原位杂交CCND1阳性对于诊断套细胞淋巴瘤具有特异性。

Objective To summarize and analyse the clinicopathological characteristics of tonsillar mantle cell lymphoma and to explore the key points of its diagnosis and differential diagnosis. Method 4 mantle cell lymphoma cases were collected and retrospective analysis as well as follow-up of their clinical and pathological material were conducted. All samples were managed with routine production and HE stain, furthermore, immunohistochemical staining and detection of CCND1 immunofluorescence in situ hybridization. Result 3 male cases and 1 female case were enrolled in our study. The age ranged from 53 to 74 years old and the median was 58 years old. There were 1 in situ mantle cell lymphoma, 1 blastic variant form of mantle cell lymphoma and 2 common form of mantle cell lymphoma. The immunohistochemical staining displayed positive CD20, CD79 a, Pax5, Cyclin D1, CD5, CD21 germinal center FDC network, CD23 germinal center FDC network, while CD3, CD45 RO, BCL2, BCL6, CD10 and TdT were negative. FISH detection showed a cracked IGH/CCND1. Patient suffering from in situ mantle cell lymphoma was first detected in early March 2014 but no treatment was implemented, and no metastasis or recrudescence occurred till 2017. The onset of the patient with blastic variant form of mantle cell lymphoma was in early November 2013, relapsed in February 2014 after two chemotherapy cycles and died in June 2014. The onset of one patient diagnosed as common form of mantle cell lymphoma was in February 2012 and was treated with 8 chemotherapy cycles and no metastasis or recrudescence was discovered till November 2017. Another patient with common form of mantle cell lymphoma was confirmed in May 2012 but no treatment was conducted, and died in August 2015. Conclusion The prognosis of tonsillar mantle cell lymphoma was better than other positions. In all tonsillar mantle cell lymphomas, the prognosis of in situ mantle cell lymphoma is the best, while prognosis of blastic variant form of mantle cell lymphoma was poor with a short lifetime. The positive results of immunohistochemical staining of Cyclin D1 and immunofluorescence in situ hybridization of CCND1 were of high specificity for the diagnosis of mantle cell lymphoma.