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中国循证儿科杂志

中国循证儿科杂志 ›› 2023, Vol. 18 ›› Issue (4): 281-285.DOI: 10.3969/j.issn.1673-5501.2023.04.007

• 论著 • 上一篇    下一篇

伴有睾丸侵犯的淋巴母细胞淋巴瘤患儿17例病例系列报告

党宛玉1,张永红,1段彦龙1,杨菁1,周春菊2,黄爽1,张梦1,李楠1,金玲1   

  1. 国家儿童医学中心，首都医科大学附属北京儿童医院北京，100045；1 儿童肿瘤中心 肿瘤内科，儿童血液病与肿瘤分子分型北京市重点实验室，儿童肿瘤国家临床重点专科，儿科重大疾病研究教育部重点实验室，2 病理科

  • 收稿日期:2023-03-04 修回日期:2023-08-11 出版日期:2023-08-25 发布日期:2023-08-25
  • 通讯作者: 金玲

Lymphoblastic lymphoma with testicular involvement in 17 children: A case series report

DANG Wanyu1, ZHANG Yonghong1, DUAN Yanlong1, YANG Jing1, ZHOU Chunju2, HUANG Shuang1, ZHANG Meng1, LI Nan1, JIN Ling1   

  1. Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing 100045, China; 1 Medical Oncology Department, Pediatric Oncology Center, Beijing Key Laboratory of Pediatric Hematology Oncology, National Key Clinical Discipline of Pediatric Oncology, Key Laboratory of Major Diseases in Children, Ministry of Education; 2 Department of Pathology

  • Received:2023-03-04 Revised:2023-08-11 Online:2023-08-25 Published:2023-08-25
  • Contact: JIN Ling, email: mouse830@126.com

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摘要： 背景：儿童原发睾丸淋巴母细胞淋巴瘤（LBL）罕见，多为继发性，既往治疗方案多采用化疗联合睾丸局部放疗，而现代治疗方案应用含大剂量甲氨蝶呤(HDMTX)等的系统化疗而不行睾丸放疗。 目的：总结伴有睾丸侵犯的LBL患儿的临床特征及应用含HDMTX的急性淋巴细胞白血病（ALL）样方案化疗的预后。 设计：病例系列报告。 方法：回顾性纳入2009年1月至2017年4月首都医科大学附属北京儿童医院（我院）收治的以肿瘤性病灶起病的伴有睾丸侵犯的LBL患儿，采用BCHNHL2009LBL方案（来自改良BFM90方案）化疗，总结其临床特征和疗效。 主要结局指标：5年总生存率（OS）和无事件生存率（EFS）。 结果:277例LBL患儿中17例(6.1％)伴有睾丸侵犯，其中TLBL 5例，BLBL 12例,发病中位年龄6.2(2.6～13.7)岁。临床分期：Ⅲ期4例，Ⅳ期13例。纵隔占位5例，CNS侵犯3例，骨髓侵犯12例(其中8例已达白血病期)，腹股沟区、盆腔、坐骨/耻骨等侵犯11例，巨大瘤灶7例。原发性睾丸淋巴瘤1例，继发性16例。双侧睾丸侵犯6例，单侧11例。诱导缓解治疗后评估睾丸瘤灶均消失。中位随访时间为103(3～129)个月，复发6例，其中睾丸复发1例，再次按标准方案治疗后完全缓解，长期无病生存；死亡2例，均为复发后死亡。5年OS和EFS分别为（87.5±8.3）％和（68.8±11.6）％，与同期LBL患儿总生存情况比较差异无统计学意义。 结论:儿童LBL发生睾丸侵犯临床症状隐匿，化疗前常规B超检查可提高诊断率，伴有腹股沟区、骨盆、盆腔侵犯者睾丸受累风险增高。经不含睾丸放疗的标准方案规律化疗后可持续完全缓解，且可保留睾丸功能。

关键词: 淋巴母细胞淋巴瘤, 睾丸肿瘤, 儿童, 抗肿瘤联合化疗方案

Abstract: Background：Primary testicular lymphoblastic lymphoma(LBL) in children is rare and mostly secondary, and the previous treatment regimens were mainly chemotherapy combined with local radiotherapy of testis. Modern treatment regimens use high dose methotrexate (HDMTX) and other systematic chemotherapy without testicular radiotherapy. Objective：To summarize the clinical features of pediatric lymphoblastic lymphoma (LBL) with testicular involvement and to investigate the clinical efficacy and prognosis of ALLlike regimen containing HDMTX. Design：Case series report. Methods：The clinical data were collected from 17 pediatric LBL patients with neoplastic lesions and testicular involvement who were treated from January 2009 to April 2017, and chemotherapy was performed with NHL2009LBL regimen (from the modified BFM90 protocol) in lymphoma treatment regimen at Beijing Children's Hospital (BCH) Affiliated to Capital Medical University. The clinical characteristics and the curative effect were analyzed. Main outcome measures：5year overall survival rate(OS) and eventfree survival rate (EFS). Results：Of 277 children with LBL in the same period, 17 were associated with testicular involvement(6.1%), including 5 cases of TLBL and 12 cases of BLBL, with a median age of 6.2(2.613.7) years old. The clinical stages were stage Ⅲ (4 cases) and stage Ⅳ (13 cases). There were 5 cases with mediastinal mass, 3 cases with central nervous system involvement, 12 cases with bone marrow involvement (8 cases of which had reached the leukemia stage), 11 cases of inguinal, pelvic, sciatic/pubic area invasion and 7 cases of giant tumor. Primary testicular lymphoma was found in 1 case and secondary in 16 cases. Bilateral testicular invasion occurred in 6 cases and unilateral testicular invasion in 11 cases. After induction remission treatment, the testicular tumor was evaluated to disappear. The median followup time was 103(3129) months. Among the 17 children, 6 cases relapsed (only 1 case of testicular relapsed who had complete remission after treatment according to the standard protocol and longterm diseasefree survival). Two cases died of recurrence. The 5year OS was (87.5±8.3)%, and the 5year EFS was (68.8±11.6)%. Compared with the overall survival rate of LBL during the same period, the difference was not statistically significant. Conclusions：The clinical symptoms of testicular involvement in pediatric LBL are hidden. Routine Bultrasound examination before chemotherapy can improve the diagnosis rate. The risk of testicular involvement is increased in patients with groin area, pelvis and pelvic invasion. Continued complete remission can be achieved with regular chemotherapy without testicular radiotherapy, and testicular function can be preserved.

Key words: Lymphoblastic lymphoma, Testicular tumor, Children, Antitumor combined chemotherapy regimen