Yang Qinglin,Guo Weihong,Zhang Weiping,et al.Intestinal infantile fibrosarcoma: a case report and literature review[J].Journal of Clinical Pediatric Surgery,2020,19(12):1107-1112.[doi:10.3969/j.issn.1671-6353.2020.12.009]
肠道婴儿型纤维肉瘤一例报道并文献复习
- Title:
- Intestinal infantile fibrosarcoma: a case report and literature review
- Keywords:
- Colonic Neoplasms; Fibrosarcoma/SU; Fibrosarcoma/ET; Treatment Outcome
- 分类号:
- R735.3+5;R730.262
- 摘要:
- 目的 探讨肠道婴儿型纤维肉瘤（infantile fibrosarcoma，IFS）的临床特征、诊治策略、病理特点及预后。方法 回顾性分析2019年4月首都医科大学附属北京儿童医院收治的1例肠道婴儿型纤维肉瘤病例的临床资料，通过PubMed和万方数据库检索国内外肠道婴儿型纤维肉瘤的相关文献，并进行数据分析。结果 患者男，1个月13天，便血3周入院，术前增强CT提示右下腹异常强化团块，部分伸入肠腔内，行腹腔镜探查并完整切除病变肠管，病理检查结果为婴儿型纤维肉瘤。术后随访6个月，复查腹部超声未见复发和转移。回顾本例及文献报道的19例肠道婴儿型纤维肉瘤，多发病于生后7 d内（16/20，80.0%），以肠穿孔（9/20，45.0%）和肠梗阻（5/20，25.0%）为主要表现，14例行开腹手术治疗，3例行腹腔镜手术治疗，2例行开腹肿瘤切除+肠造瘘术，1例行开腹手术切除+二次手术+辅助化疗。病理学诊断结合组织学、免疫组化和分子诊断结果，发现肠道IFS的ETV6-NTRK3异位阳性率为46.1%（6/13）。所有患者预后良好。结论 肠道婴儿型纤维肉瘤是一种罕见的软组织肉瘤，多于新生儿早期发病，需结合患者病情选择合适的手术方案，完整切除后整体预后良好。
- Abstract:
- Objective To explore the clinical manifestations,pathological features,treatments and outcomes of intestinal infantile fibrosarcoma (IFS).Methods In April 2019,clinical data were retrospectively analyzed for one case of intestinal infantile fibrosarcoma.And the relevant literature reports were retrieved through the databases of PubMed and Wanfang.Results One 43-day-old boy was hospitalized for 3-week hematochezia.Preoperative contrast enhanced computed tomography (CT) hinted at a heterogeneously intensified mass in right lower abdomen with partial extension into enteric cavity.Laparoscopic exploration was performed and lesion completely resected.Pathological diagnosis confirmed IFS.During a follow-up period of 6 months,there was no sign of relapse or transformation on abdomen ultrasound.A total of 19 IFS cases were reported.Most cases (16/20,80.0%) occurred within the first week after birth.There were intestinal enterobrosis (9/20,45.0%) and intestinal obstruction (5/20,25.0%).Surgery was effective while adjuvant chemotherapy was required for relapse.A definite diagnosis was dependent upon histology,immunohistochemistry and molecular diagnostics.Ectopia of ETV6-NTRK3 was detected in 46.1%(6/13) of intestinal IFS.The overall prognosis was decent.Conclusion Intestional IFS is a rare soft-tissue sarcoma usually occurring during early neonatal period.Proper surgical approach should be employed for complete lesion resection.And the overall prognosis is excellent.
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备注/Memo
收稿日期:2019-10-08。
基金项目:北京市医院管理局"扬帆计划"（编号：ZYLX201709）：北京市医院管理局"登峰"人才培养计划（编号：DFL20151102）
通讯作者:郭卫红,Email:guo_weihong@163.com;张潍平,Email:zhangwpp@163.com