Sun Junjie,Shu Man,Gao Pengfei,et al.Clinical analysis of diagnosing and treating gastrointestinal stromal tumors in children[J].Journal of Clinical Pediatric Surgery,2019,18(08):707-710.[doi:10.3969/j.issn.1671-6353.2019.08.018]
儿童胃肠道间质瘤3例报告并文献复习
- Title:
- Clinical analysis of diagnosing and treating gastrointestinal stromal tumors in children
- 分类号:
- R735.2;R735.3
- 摘要:
- 目的 分析儿童胃肠道间质瘤的临床特点并总结其诊治策略。方法 回顾性分析近5年中山大学附属第一医院收治的3例胃肠道间质瘤患儿的临床资料,并结合国内外资料进行文献复习。结果 3例患儿,男童1例,女童2例,分别为9岁、11岁和15.6岁;临床表现为头晕乏力、面色苍白、排黑便及腹痛等;胃镜可见胃部黏膜下肿物并出血,腹部CT提示胃小弯侧软组织占位;手术方法为胃部分切除或转移瘤切除术;3例患儿术后均口服酪氨酸激酶抑制剂治疗;随访10~48个月,1例复发,并再次接受手术,其余2例无复发。结论 儿童胃肠道间质瘤临床极为罕见,以野生型为主;胃镜及腹部CT检查有助于诊断,治疗以手术为主综合治疗。
- Abstract:
- Objective To summarize the clinical characteristics,diagnosis and treatment of gastrointestinal stromal tumors in children.Methods Three children diagnosed as gastrointestinal stromal tumors were admitted over the last five years.Retrospective analysis was performed for clinical data of 3 cases along with a review of related literature studies.Results There were 1 boy and 2 girls with a median age of 12 years.Their clinical manifestations included dizziness,fatigue,pale complexion,melena and abdominal pain.Endoscopy revealed submucosal tumor with hemorrhage and abdominal computer tomography (CT) hinted at an occupation of soft mass at the lesser gastric curvature.Surgical options were partial gastrectomy and metastatic tumor resection;All 3 cases received tyrosine kinase inhibitors post-operation.During a follow-up period of 10-48 months,1 recurrent case was re-operated while the remainder was uneventful.Conclusion Pediatric gastrointestinal stromal tumors are extremely rare and most cases belong to wild-type.Both gastroscopy and abdominal CT are helpful for reaching a definite diagnosis.Comprehensive therapy is essential and surgery remains a major treatment.
参考文献/References:
1 Cianci P,Luini C,Marinoni M,et al.Pediatric GIST presenting as anemia[J].Pediatr Hematol Oncol,2017,34(5):343-347.DOI:10.1080/08880018.2017.1396387.
2 Tran S,Dingeldein M,Mengshol SC,et al.Incidental GIST after appendectomy in a pediatric patient:a first instance and review of pediatric patients with CD117 confirmed GISTs[J].Pediatric Surg Int,2014,30(4):457-466.DOI:10.1007/s00383-013-3432-3.
3 Kurucu N,Sari N,Celasun B,et al.Gastrointestinal stromal tumor in a newborn diagnosed in prenatal period:a case report and review of literature[J].J Pediatr Hematol Oncol,2014,36(8):649-652.DOI:10.1097/MPH.0b013e3182a8f73f.
4 Bhatt NR,Collins D,Crotty P,et al.Prognosis and management of adult wild type gastrointestinal stromal tumours (GISTs):A pooled analysis and review of literature[J].Surg Oncol,2016,25(3):152-157.DOI:10.1016/j.suronc.2016.05.003.
5 Weldon CB,Madenci AL,Boikos SA,et al.Surgical management of wild-type gastrointestinal stromal tumors:a report from the National Institutes of Health Pediatric and Wildtype GIST Clinic[J].J Clin Oncol,2017,35(5):523-528.DOI:10.1200/JCO.2016.68.6733.
6 Lima M,Gargano T,Ruggeri G,et al.Laparoscopic resection of a rare gastrointestinal stromal tumor in children[J].Springerplus,2015,4:73.DOI:10.1186/s40064-015-0850-9.
7 Mullassery D,Weldon CB.Pediatric/"Wildtype" gastrointestinal stromal tumors[J].Semin Pediatr Surg,2016,25(5):305-310.DOI:10.1053/j.sempedsurg.2016.09.004.
8 Nannini M,Astolfi A,Urbini M,et al.Integrated genomic study of quadruple-WT GIST (KIT/PDGFRA/SDH/RAS pathway wild-type GIST)[J].BMC Cancer,2014,14:685.DOI:10.1186/1471-2407-14-685.
9 Corless CL,Fletcher JA,Heinrich MC.Biology of gastrointestinal stromal tumors[J].J Clin Oncol,2004,22(18):3813-3825.DOI:10.1200/JCO.2004.05.140.
10 Kalfusova A,Kodet R.Molecular mechanisms of primary and secondary resistance,molecular-genetic features and characteristics of KIT/PDGFRA non-mutated GISTs[J].Cesk Patol,2017,53(4):167-173.
11 Boikos SA,Stratakis CA.The genetic landscape of gastrointestinal stromal tumor lacking KIT and PDGFRA mutations[J].Endocrine,2014,47(2):401-408.DOI:10.1007/s12020-014-0346-3.
12 Killian JK,Miettinen M,Walker RL,et al.Recurrent epimutation of SDHC in gastrointestinal stromal tumors[J].Sci Transl Med,2014,6(268):268ra177.DOI:10.1126/scitranslmed.3009961.
13 Agaram NP,Laquaglia MP,Ustun B,et al.Molecular characterization of pediatric gastrointestinal stromal tumors[J].Clin Cancer Res,2008,14(10):3204-3215.DOI:10.1158/1078-0432.CCR-07-1984.
14 Janeway KA,Albritton KH,Van Den Abbeele AD,et al.Sunitinib treatment in pediatric patients with advanced GIST following failure of imatinib[J].Pediatr Blood Cancer,2009,52(7):767-771.DOI:10.1002/pbc.21909.
相似文献/References:
[1]杜朝峻,丁力,胡英超,等.手术治疗婴幼儿法洛四联症83例[J].临床小儿外科杂志,2010,9(06):413.
DU Chao jun,DING Li,HU Ying chao,et al.Surgical treatment of Tetralogy of Fallot in infancy: Experiences of 83 cases.[J].Journal of Clinical Pediatric Surgery,2010,9(08):413.
[2]常绘文,沈振亚.右腋下小切口在儿童心内手术中的应用[J].临床小儿外科杂志,2010,9(06):439.
[3]王勇,汤绍涛,毛永忠,等.腔镜下手术治疗小儿膈肌疾病31例[J].临床小儿外科杂志,2010,9(06):441.
[4]欧阳帆,张治明,曾宾,等.七氟醚复合髂腹股沟-髂腹下神经阻滞用于小儿腹股沟手术[J].临床小儿外科杂志,2010,9(06):466.
[5]徐华,赵陶丽.瑞芬太尼对小儿等离子刀扁桃体腺样体切除术应激反应的影响[J].临床小儿外科杂志,2010,9(06):468.
[6]周辉霞孙宁马立飞孟浩谢华伟申州陶天周晓光黄澄如李宏召张旭.腹腔镜下重复肾半肾切除术手术路径探讨[J].临床小儿外科杂志,2011,10(01):0.
ZHOU Hui xia,SU Ning,MA Li fei,et al.Which laparoscopic surgery approach is better for a nonfunctioning moiety in a duplex kidney in infants and children[J].Journal of Clinical Pediatric Surgery,2011,10(08):0.
[7]蒋欣李箭刘利君彭明惺唐学阳.儿童单纯性膝关节骨软骨骨折的关节镜治疗[J].临床小儿外科杂志,2011,10(01):0.
JIANG Xin,LI Jian,LIU Li jun,et al.Arthroscopic therapy of osteochondral fractures of the Knee in children.[J].Journal of Clinical Pediatric Surgery,2011,10(08):0.
[8]王伟程少文彭磊林忠勤张伟陈庆玉寇冬权.弹性髓内钉在儿童肱骨骨折中的应用[J].临床小儿外科杂志,2011,10(01):0.
WANG Wei,CHENG Shaowen,PENG Lei,et al.Application of elastic intramedullary nailing in pediatric humeral fractures.[J].Journal of Clinical Pediatric Surgery,2011,10(08):0.
[9]寿铁军李勇马能强龚晟.腹腔镜手术治疗小儿腹股沟斜疝1582例[J].临床小儿外科杂志,2011,10(01):0.
[10]吴臻斐陈聪德林进汉刘平李仲荣陈肖鸣.小儿急性肠套叠肠坏死相关因素分析[J].临床小儿外科杂志,2011,10(01):0.
备注/Memo
收稿日期:2018-08-30。
通讯作者:高鹏飞,Email:gaofei.06@163.com