Feng Dan,Li Zheng.Analysis of difficulties in the diagnosis and treatment of gastrointestinal lymphoma in children with intussusception as an initial presentation[J].Journal of Clinical Pediatric Surgery,2026,(02):159-164.[doi:10.3760/cma.j.cn101785-202502034]
以肠套叠为首发表现的儿童胃肠道淋巴瘤诊治难点分析
- Title:
- Analysis of difficulties in the diagnosis and treatment of gastrointestinal lymphoma in children with intussusception as an initial presentation
- Keywords:
- Lymphoma; Gastrointestinal Tract; Intussusception; Diagnosis; Therapy; Child
- 摘要:
- 目的 探讨以肠套叠为首发表现的儿童胃肠道淋巴瘤的临床特征及诊治难点,以提升该病的诊治水平。方法 本研究为回顾性病例系列分析,回顾性收集2009年3月31日至2023年7月31日江西省儿童医院普外科收治的33例以肠套叠为首发表现的儿童胃肠道淋巴瘤患者临床资料,并于2025年2月前完成随访。总结患儿年龄、性别、发病时长、实验室检查、影像学检查、病理结果、治疗及预后情况等。结果 33例中,男26例、女7例;发病年龄1~13岁,其中5岁7例;患儿主要因腹痛就诊,其中27例就诊时发病时长超过48 h,仅6例在48 h内就诊。4例血清乳酸脱氢酶值(lactate dehydrogenase,LDH)高于1 299 U/L,其中3例术中肉眼见巨大瘤灶、腹腔多发转移灶,1例术后病理结果提示阑尾及肠系膜淋巴结转移,2例骨髓穿刺检查结果提示骨髓转移。9例年龄＜5岁的患儿初诊时超声检查除肠套叠外,未见其他异常,其中6例术中复位肠套叠后仅表现为肠壁肿胀僵硬。24例年龄≥5岁的患儿中,10例初诊超声发现低回声包块,术中均见局部肿物形成。33例均行手术,其中4例行腹腔镜手术(2例因肠套叠复位困难而中转开腹手术,2例因腹腔镜手术后肠套叠复发再次行开腹手术发现肠壁存在异常肿胀僵硬),术后病理结果均为非霍奇金淋巴瘤(non-Hodgkin lymphoma,NHL)。33例中,7例失访;26例获随访,其中21例接受化疗(20例完全缓解,1例复发),5例术后未继续治疗(3例随访至今未见异常,2例病情迅速进展死亡)。结论 5岁是儿童胃肠道淋巴瘤的高发年龄。部分肿瘤仅表现为肠壁肿胀僵硬,是影像学检查及腹腔镜手术中诊断困难的主要原因。当LDH高于正常上限值5倍时,可能伴有巨大瘤灶、腹腔转移灶和(或)骨髓转移。
- Abstract:
- Objective To explore the clinical characteristics of gastrointestinal lymphoma (GIL) in children with intussusception so as to boost the levels of its diagnosis and treatment. Methods The relevant clinical data were retrospectively reviewed for 33 GIL children with intussusception operated from March 31,2009 to July 31,2023.Follow-ups were conducted until February 2025.General profiles (gender,onset age & duration of disease),laboratory parameters,imaging studies,pathological examinations,treatments and outcomes were recorded. Results Among them,there were 26 boys and 7 girls with an onset age of 1 to 13 year.Seven cases were aged 5 year.Abdominal pain occurred over 48 h (n=27) and within 48 h (n=6).The level of lactate dehydrogenase (LDH) surpassed 1299 U/L(n=4).There were large tumors with multiple metastatic lesions (n=4).In another case.metastasis in mesenteric lymph node and appendix were detected by postoperative pathology examination.And 2/4 cases revealed bone marrow (BM) metastasis by BM aspiration.Nine of them were aged under 5 year.Initial ultrasonic examinations (excluding intussusception) revealed no abnormalities.And 6/9 cases hinted at merely swelling and stiffness of intestinal wall during surgery.And 24 cases were aged 5 years or above.Local masses were detected in all of them during surgery.Hypoechoic ultrasonic masses were reported at an initial diagnosis.Surgical procedures included laparoscopy (n=4),conversion into open surgery due to difficulties of intussusception reduction (n=2) and reoperation for recurrent intussusception due to abnormal intestinal wall swelling and rigidity (n=2). Postoperative pathological examinations confirmed non-Hodgkin lymphoma.Seven cases became lost to follow-ups.In the remainders,21 received postoperative chemotherapy was offered (n=21) and there were complete remission (n=20) and relapse (n=1).In another 5 cases,there were no abnormalities (n=3) and death from rapid disease progression (n=2). Conclusions Age 5 has been a high incidence age of GIL in children.When tumor is manifested merely as swelling and stiffness of intestinal wall,it is a major cause of diagnostic difficulties through imaging and laparoscopy.When LDH value is 5 folds above its upper limit of normal,there are also accompanied by large tumor lesion,multiple metastatic lesions and (or) BM metastasis.
参考文献/References:
[1] 张永红.儿童非霍奇金淋巴瘤的生物学和病理学[M]//Weinstein HJ,Hudson MM,Link MP.儿童淋巴瘤.张永红,段彦龙,张蕊,译.北京:人民卫生出版社,2009:79-104. Zhang YH.Pediatric Biology and Pathology of non-Hodgkin’s Lymphoma[M]//Weinstein HJ,Hudson MM,Link MP.Pediatric Lymphomas.Beijing:People’s Medical Publishing House,2009:79-104.
[2] 周峻,陈芳林,王浩,等.肠套叠为首发表现儿童原发性胃肠道淋巴瘤临床特征分析[J].中华肿瘤防治杂志,2015,22(19):1561-1565.DOI:10.16073/j.cnki.cjcpt.2015.19.014. Zhou J,Chen FL,Wang H,et al.Clinical cases analysis of primary gastrointestinal lymphoma with intussusception as the first presentation[J].Chin J Cancer Prev Treat,2015,22(19):1561-1565.DOI:10.16073/j.cnki.cjcpt.2015.19.014.
[3] 黄召,刘登辉.儿童原发性肠道淋巴瘤继发肠套叠的手术治疗[J].临床小儿外科杂志,2023,22(2):163-167.DOI:10.3760/cma.j.cn101785-202204042-013. Huang Z,Liu DH.Surgical treatment on secondary intussusception with primary intestinal lymphoma in children[J].J Clin Ped Sur,2023,22(2):163-167.DOI:10.3760/cma.j.cn101785-202204042-013.
[4] 杨文萍,朱才娣,宫丽平,等.儿童腹腔非霍奇金B细胞淋巴瘤的临床病理及免疫表型分析[J].中华病理学杂志,2009,38(11):759-764.DOI:10.3760/cma.j.issn.0529-5807.2009.11.010. Yang WP,Zhu CD,Gong LP,et al.Clinicopathologic and immunohistochemical study of intra-abdominal non-Hodgkin B-cell lymphoma occurring in children[J].Chin J Pathol,2009,38(11):759-764.DOI:10.3760/cma.j.issn.0529-5807.2009.11.010.
[5] Gupta H,Davidoff AM,Pui CH,et al.Clinical implications and surgical management of intussusception in pediatric patients with Burkitt lymphoma[J].J Pediatr Surg,2007,42(6):998-1001.DOI:10.1016/j.jpedsurg.2007.01.033.
[6] Burkhardt B,Zimmermann M,Oschlies I,et al.The impact of age and gender on biology,clinical features and treatment outcome of non-Hodgkin lymphoma in childhood and adolescence[J].Br J Haematol,2005,131(1):39-49.DOI:10.1111/j.1365-2141.2005.05735.x.
[7] 董娟,秦鸣,陈玉龙.儿童胃肠道Burkitt淋巴瘤的超声特点及漏误诊分析[J].中国超声医学杂志,2022,38(4):470-472.DOI:10.3969/j.issn.1002-0101.2022.04.034. Dong J,Qin M,Chen YL.Analysis of ultrasonic characteristics and missed diagnosis of gastrointestinal Burkitt lymphoma in children[J].Chin J Ultrasound Med,2022,38(4):470-472.DOI:10.3969/j.issn.1002-0101.2022.04.034.
[8] Worch J,Rohde M,Burkhardt B.Mature B-cell lymphoma and leukemia in children and adolescents-review of standard chemotherapy regimen and perspectives[J].Pediatr Hematol Oncol,2013,30(6):465-483.DOI:10.3109/08880018.2013.783891.
[9] Reiter A,Klapper W.Recent advances in the understanding and management of diffuse large B-cell lymphoma in children[J].Br J Haematol,2008,142(3):329-347.DOI:10.1111/j.1365-2141.2008.06988.x.
[10] 黄爽,金玲,杨菁,等.复发儿童B细胞淋巴瘤19例临床病理特征及治疗预后分析[J].中华儿科杂志,2017,55(10):748-753.DOI:10.3760/cma.j.issn.0578-1310.2017.10.007. Huang S,Jin L,Yang J,et al.Clinical pathologic characteristics and treatment outcomes of 19 relapsed pediatric B-cell lymphoma[J].Chin J Pediatr,2017,55(10):748-753.DOI:10.3760/cma.j.issn.0578-1310.2017.10.007.
[11] 张梦,金玲,杨菁,等.儿童伯基特淋巴瘤186例临床特征及疗效分析[J].中华儿科杂志,2018,56(8):605-610.DOI:10.3760/cma.j.issn.0578-1310.2018.08.010. Zhang M,Jin L,Yang J,et al.Clinical and prognostic analysis of 186 children with Burkitt’s lymphoma[J].Chin J Pediatr,2018,56(8):605-610.DOI:10.3760/cma.j.issn.0578-1310.2018.08.010.
[12] 中华医学会儿科学分会肿瘤学组,中华医学会儿科学分会血液学组,中国抗癌协会小儿肿瘤专业委员会,等.儿童和青少年侵袭性成熟B细胞非霍奇金淋巴瘤诊疗专家共识[J].中华儿科杂志,2020,58(10):790-795.DOI:10.3760/cma.j.cn112140-20200723-00745. The Subspecialty Group of Oncology,the Society of Pediatrics,Chinese Medical Association,The Subspecialty Group of Hematology,the Society of Pediatrics,Chinese Medical Association,Committee of Pediatrics,Chinese Anti-Cancer Association,et al.Experts consensus on current management of children and adolescents with aggressive mature B-cell non-Hodgkin lymphoma[J].Chin J Pediatr,2020,58(10):790-795.DOI:10.3760/cma.j.cn112140-20200723-00745.
[13] Gualco G,Queiroga EM,Weiss LM,et al.Frequent expression of multiple myeloma 1/interferon regulatory factor 4 in Burkitt lymphoma[J].Hum Pathol,2009,40(4):565-571.DOI:10.1016/j.humpath.2008.07.021.
[14] Dobashi A,Tsuyama N,Asaka R,et al.Frequent BCOR aberrations in extranodal NK/T-cell lymphoma,nasal type[J].Genes Chromosomes Cancer,2016,55(5):460-471.DOI:10.1002/gcc.22348.
[15] 黄慧,樊金星,吴艳,等.儿童散发性伯基特淋巴瘤中MUM1的异常表达及其临床病理特征分析[J].实用癌症杂志,2021,36(9):1453-1456.DOI:10.3969/j.issn.1001-5930.2021.09.016. Huang H,Fan JX,Wu Y,et al.Aberrant expression of MUM1 and clinicopathological features in pediatric sporadic Burkitt lymphoma[J].Pract J Cancer,2021,36(9):1453-1456.DOI:10.3969/j.issn.1001-5930.2021.09.016.
[16] 王悦冰,赵晓甦.p53基因在血液肿瘤中的临床意义[J].白血病·淋巴瘤,2024,33(4):245-248.DOI:10.3760/cma.j.cn115356-20230718-00006. Wang YB,Zhao XS.Clinical significances of p53 gene in hematologic neoplasms[J].J Leuk Lymphoma,2024,33(4):245-248.DOI:10.3760/cma.j.cn115356-20230718-00006.
[17] Bhavsar S,Liu YC,Gibson SE,et al.Mutational landscape of TdT+ large B-cell lymphomas supports their distinction from B-lymphoblastic neoplasms:a multiparameter study of a rare and aggressive entity[J].Am J Surg Pathol,2022,46(1):71-82.DOI:10.1097/PAS.0000000000001750.
[18] Qiu LQ,Xu J,Lin P,et al.Unique pathologic features and gene expression signatures distinguish blastoid high-grade B-cell lymphoma from B-acute lymphoblastic leukemia/lymphoma[J].Haematologica,2023,108(3):895-899.DOI:10.3324/haematol.2022.281646.
[19] 代洁,谭燕勤,马越,等.TdT阳性大B细胞淋巴瘤1例[J].肿瘤预防与治疗,2023,36(9):759-762.DOI:10.3969/j.issn.1674-0904.2023.09.007. Dai J,Tan YQ,Ma Y,et al.TdT-Positive large B-cell lymphoma:one case report[J].J Cancer Control Treat,2023,36(9):759-762.DOI:10.3969/j.issn.1674-0904.2023.09.007.
[20] Minard-Colin V,Aupérin A,Pillon M,et al.Rituximab for high-risk,mature B-cell non-Hodgkin’s lymphoma in children[J].N Engl J Med,2020,382(23):2207-2219.DOI:10.1056/NEJMoa1915315.
[21] Moleti ML,Testi AM,Foà R.Treatment of relapsed/refractory paediatric aggressive B-cell non-Hodgkin lymphoma[J].Br J Haematol,2020,189(5):826-843.DOI:10.1111/bjh.16461.
相似文献/References:
[1]杨姣 周扬莹 祝晟 张屹 傅春燕 陈晨 周建华.小儿原发性胃肠道非霍奇金淋巴瘤的临床病理分析[J].临床小儿外科杂志,2011,10(03):193.
[J].Journal of Clinical Pediatric Surgery,2011,10(02):193.
[2]侯大为 陈永卫 郭卫红 李樱子.新生儿肠动力障碍症的治疗探讨 [J].临床小儿外科杂志,2011,10(03):212.
[J].Journal of Clinical Pediatric Surgery,2011,10(02):212.
[3]赵萍 刘翔 左伟 高威 张燕敏 陈晓莉 沈浩. 新生儿消化道穿孔55例临床分析 [J].临床小儿外科杂志,2011,10(06):470.
[J].Journal of Clinical Pediatric Surgery,2011,10(02):470.
[4]伍光春 李殊明 金科 陈桦 罗韦华 刘慧亚. 小儿腹腔非霍奇金淋巴瘤的CT影像分析[J].临床小儿外科杂志,2012,11(01):47.
[5]武鹏 陈肖鸣. 小儿肠道异物手术治疗9例分析[J].临床小儿外科杂志,2014,13(06):537.
[6]陈巍,王晓曼,刘婷婷,等.儿童消化道异物21例手术治疗分析[J].临床小儿外科杂志,2021,20(08):778.[doi:10.12260/lcxewkzz.2021.08.014]
Chen Wei,Wang Xiaoman,Liu Tingting,et al.Clinical experience of ingested intraabdominal foreign bodies in children requiring surgical intervention[J].Journal of Clinical Pediatric Surgery,2021,20(02):778.[doi:10.12260/lcxewkzz.2021.08.014]
[7]刘业根,李刚龙,罗秋妹,等.小儿消化道磁性金属异物6例及文献复习[J].临床小儿外科杂志,2022,21(12):1185.[doi:10.3760/cma.j.cn101785-202104060-016]
Liu Yegen,Li Ganglong,Luo Qiumei,et al.Accidental ingestion of metallic magnetic foreign bodies:a report of six cases with a literature review[J].Journal of Clinical Pediatric Surgery,2022,21(02):1185.[doi:10.3760/cma.j.cn101785-202104060-016]
[8]黄召,刘登辉.儿童原发性肠道淋巴瘤继发肠套叠的手术治疗[J].临床小儿外科杂志,2023,22(02):163.[doi:10.3760/cma.j.cn101785-202202018-013]
Huang Zhao,Liu Denghui.Surgical treatment on secondary intussusception with primary intestinal lymphoma in children[J].Journal of Clinical Pediatric Surgery,2023,22(02):163.[doi:10.3760/cma.j.cn101785-202202018-013]
[9]文佳冰,李勇,肖雅玲,等.淋巴结活检在儿童淋巴瘤相关噬血细胞综合征筛查中的应用[J].临床小儿外科杂志,2023,22(03):267.[doi:10.3760/cma.j.cn101785-202207057-012]
Wen Jiabing,Li Yong,Xiao Yaling,et al.Value of lymph node biopsy in screening children with lymphoma-associated hemophagocytic syndrome[J].Journal of Clinical Pediatric Surgery,2023,22(02):267.[doi:10.3760/cma.j.cn101785-202207057-012]
[10]钱波,莫绪明,戚继荣,等.小儿胸部实体进展期恶性肿瘤手术策略的临床研究[J].临床小儿外科杂志,2025,(05):419.[doi:10.3760/cma.j.cn101785-202501066-005]
Qian Bo,Mo Xuming,Qi Jirong,et al.Surgical strategies for advanced malignant chest tumors in children[J].Journal of Clinical Pediatric Surgery,2025,(02):419.[doi:10.3760/cma.j.cn101785-202501066-005]
备注/Memo
收稿日期:2025-2-18。
通讯作者:李争,Email:lizheng0412@126.com