腹内型侵袭性纤维瘤的临床特征  

The clinical characteristics of intra-abdominal aggressive fibromatosis

作　　者：黄远健 胥子玮 HUANG Yuanjian;XU Ziwei(Department of Colorectal Surgery,The First Affiliated Hospital of Nanjing Medical University,Nanjing 210029,China)

机构地区：[1]南京医科大学第一附属医院结直肠外科,江苏南京210029

出　　处：《中国肿瘤外科杂志》2025年第1期75-79,86,共6页Chinese Journal of Surgical Oncology

基　　金：南京医科大学第一附属医院国家自然科学基金青年基金培育计划(PY202408)。

摘　　要：目的汇总分析腹内型侵袭性纤维瘤(IAF)患者的常见临床特征,以提高对该疾病的认识和诊疗水平。方法建立回顾性单臂队列,纳入2017年至2023年于南京医科大学第一附属医院接受手术的32例IAF患者,对其一般情况、影像学特征、手术情况、病理学特征和预后情况进行汇总分析。结果32例IAF患者的中位年龄为43岁,男性占多数(56.3%),首发症状多为腹痛(50.0%),多有胃癌手术史(18.8%),单发肿瘤最为常见(81.2%),最常累及小肠及其系膜的中腹部区域(56.2%),肿瘤大小不一,小至5 cm以内(46.9%),大可至20 cm以上(3.1%)。影像学上,肿瘤多呈类圆形,常与周围组织分界不清,CT平扫多为稍低密度,MRI的T1WI相为等或稍低信号,T2WI相为稍高信号,CT和MRI增强扫描后均可见不均匀强化。绝大部分患者完成了R0切除(90.6%),术后病理诊断需依赖β-catenin、Vimentin、SMA、S-100、CD117等免疫组化染色。接受R0切除的患者总复发率为8%。结论IAF是一种罕见肿瘤,诊断需依赖详细的病史询问、影像学检查和病理学免疫组化检查。对于IAF患者,应做好全面评估,选择最佳治疗方案,从而改善患者预后。Objective To summarize and analyze the common clinical characteristics of patients with intra-abdominal aggressive fibromatosis(IAF),in order to enhance the understanding,diagnostic,and therapeutic level of this disease.Methods Establish a retrospective single-arm cohort,including 32 IAF patients who underwent surgery at the First Affiliated Hospital of Nanjing Medical University from 2017 to 2023.A summary analysis was conducted on their general conditions,imaging characteristics,surgical conditions,pathological features,and prognostics.Results The median age of the 32 IAF patients was 43 years,with a male predominance(56.3%).The most common initial symptom was abdominal pain(50.0%),and many had a history of gastric cancer surgery(18.8%).Solitary tumors were the most frequent(81.2%),typically located in the mid-abdomen involving the small intestine and its mesentery(56.2%).Tumor sizes varied from less than 5 cm(46.9%)to over 20 cm(3.1%).On imaging,tumors often appeared round-like with unclear boundaries from surrounding tissues.On CT scans,tumors predominantly showed slightly low density,while MRI demonstrated isointense or slightly low signals on T1WI and slightly high signals on T2WI.Both CT and MRI enhanced scans revealed heterogeneous enhancement.Most patients achieved R0 resection(90.6%).Postoperative pathological diagnosis relied on immunohistochemical staining includingβ-catenin,Vimentin,SMA,S-100,and CD117.The overall recurrence rate after R0 resection was 8%.Conclusions IAF is a rare tumor whose diagnosis depends on a detailed medical history,imaging,and immunohistochemical pathology examination.Comprehensive evaluation should be performed for IAF patients to select the optimal treatment strategy,thereby improving patient outcomes.

关 键 词：侵袭性纤维瘤 硬纤维瘤 腹内型 临床特征 回顾性研究 

分 类 号：R735[医药卫生—肿瘤]