摘要
目的:总结分析儿童型滤泡淋巴瘤(PTFL)的病理诊断、临床特征、治疗方法及转归。方法:回顾性总结2017年2月至2023年2月河南省肿瘤医院收治的9例PTFL患儿的临床资料,分析其病理、临床特征、治疗方法及随访结果。结果:9例患儿发病年龄为6~18岁,均为男童。临床均表现为头颈部局部无痛性淋巴结肿大,临床分期为Ⅰ~Ⅱ期。PTFL组织形态学特征与经典型的滤泡淋巴瘤(FL)相似,大部分滤泡生发中心扩大,套区消失,中心母细胞易见,组织学分级多为Ⅲ级,可伴“星空”现象。B细胞克隆性重排(BCR)可见单克隆峰。免疫组织化学(IHC)染色示CD20、CD10、Bcl-6阳性,Bcl-2、C-myc阴性,Ki-67为70%~95%。荧光原位杂交(FISH)检测t(14,18)阴性,Bcl-2易位、C-myc易位阴性。6例单纯手术切除,3例手术切除联合化疗。截至2023年2月,随访时间45~72个月,所有患儿均存活,无一例复发,均处于完全缓解状态。结论:PTFL主要以青少年男性发病为主,病理表现为组织学高级别状态,高增殖指数,缺乏t(14;18)、Bcl-2易位及Bcl-2表达。以局部手术切除治疗为主,预后良好。
Objective To summarize the pathological diagnosis,clinical features,treatment methods and outcomes of pediatric-type follicular lymphoma(PTFL).Methods Clinical data including the pathology,clinical features,treatment methods,and follow-up results of 9 PTFL patients admitted to Henan Cancer Hospital from February 2017 to February 2023 were analyzed retrospectively.Results The age of onset in 9 children was 6 to 18 years,all the patients were males.The clinical manifestation was local painless lymph node enlargement in the head and neck,with a stage ofⅠ-Ⅱ.The histomorphological characteristics of PTFL were similar to those of classic follicular lymphoma(FL).The germinal center of most follicles were enlarged,the mantle zone disappeared,centroblasts were easily visible,and the histological grade were mostly gradeⅢ,which may be accompanied by the"starry sky"phenomenon.Monoclonal peaks can be seen in B cell clonal rearrangements(BCR).Immunohistochemistry(IHC)showed CD20 positive,CD10 positive,Bcl-6 positive,Bcl-2 negative,C-myc negative,and Ki-67 was 70%-95%.Fluorescence in situ hybridization(FISH)test was negative for t(14,18),Bcl-2 translocation,and C-myc translocation.Six cases underwent surgical resection,and 3 cases underwent surgical resection combined with chemotherapy.Up to February 2023,with a follow-up time of 45 to 72 months,all children survived without any recurrence and were in a complete remission state.Conclusions PTFL is mainly characterized by adolescent male onset,with early clinical manifestations and pathological manifestations of high-level histological status,high proliferation index,and lack of t(14;18)/Bcl-2 translocation and Bcl-2 expression.It is mainly treated by localized surgical excision and has a good prognosis.
作者
李钢苹
张笛
王勇奇
李子夜
袁芳芳
李明会
王璐
杜建伟
张丽娜
李玉富
魏旭东
符粤文
Li Gangping;Zhang Di;Wang Yongqi;Li Ziye;Yuan Fangfang;Li Minghui;Wang Lu;Du Jianwei;Zhang Lina;Li Yufu;Wei Xudong;Fu Yuewen(Department of Hematology,the Affiliated Cancer Hospital of Zhengzhou University,Henan Cancer Hospital,Zhengzhou 450008,China;Medical Record Department,the Affiliated Cancer Hospital of Zhengzhou University,Henan Cancer Hospital,Zhengzhou 450008,China;Department of Pathology,the Affiliated Cancer Hospital of Zhengzhou University,Henan Cancer Hospital,Zhengzhou 450008,China)
出处
《中华儿科杂志》
CAS
CSCD
北大核心
2023年第12期1129-1132,共4页
Chinese Journal of Pediatrics
基金
河南省科技攻关计划联合共建项目(LHGJ20220188)
河南省中青年卫生健康科技创新优秀青年人才培养项目(YXKC2022046)。
关键词
淋巴瘤
滤泡性
病理学
预后
Lymphoma,follicular
Pathology
Prognosis
