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肠系膜侵袭性纤维瘤病:病例报告及文献复习OA

Mesenteric aggressive fibromatosis:A case report and literature review

中文摘要英文摘要

目的 通过报道1 例肠系膜侵袭性纤维瘤病(aggressive fibromatosis,AF)包绕肠系膜上动脉病例并复习文献,探讨其诊治思路及进展.方法 回顾性分析 1 例 40 岁女性患者,主诉腹部肿块伴腹胀2 月余,查体于左上腹触及质地偏韧、活动度差的巨大包块,影像学提示腹腔巨大占位并包绕肠系膜上动脉的肠系膜 AF 患者的临床资料,整理其症状、实验室及影像学检查结果、多学科术前评估意见、手术过程、病理结果及随访情况,并汇总同类病例文献,探讨其诊治方法.结果 本例经多学科会诊后行手术治疗,切除肿块及部分空肠,重建肠系膜上动脉.术后病理确诊为肠系膜 AF,切缘阴性.随访6 个月,肠系膜上动脉血运良好,无肿瘤复发转移.汇总相关文献(9 例),共 10 例肠系膜AF 病例,其中男性7 例,女性3 例;平均年龄43.2(27~69)岁.发生部位:小肠系膜5 例,回盲部肠系膜1 例,结直肠系膜2 例,不明部位肠系膜2 例,均行手术治疗.术后 1 天死亡 1 例,预后不明 2 例,余者随访预后尚可.结论 AF 是罕见的交界性软组织肿瘤,腹内型 AF 少见且诊疗困难.不同类型、大小的肠系膜 AF 应个体化制定诊疗方案;对于累及 SMA 的肠系膜 AF,实施保留器官的根治性切除联合血管重建方案安全可行.

Objective To explore the diagnosis,treatment,and research progress of mesenteric aggressive fibromatosis(AF)by reporting a case of AF encasing the superior mesenteric artery(SMA)and reviewing the literature.Methods A retrospective analysis was conducted on the clinical data of a 40-year-old female patient with mesenteric AF,who presented with an abdominal mass and abdominal distension for more than 2 months.Physical examination revealed a firm,poorly mobile,massive mass in the left upper abdomen.Imaging revealed a massive intra-abdominal space-occupying lesion encasing the SMA.The clinical manifestations,laboratory and imaging findings,multidisciplinary preoperative evaluation,surgical procedures,pathological results,and follow-up data were systematically collected.Additionally,relevant literature was retrieved and similar cases were summarized to explore the diagnostic and therapeutic approaches for this condition.Results The patient underwent surgical resection of the mass and partial jejunum combined with SMA reconstruction after multidisciplinary evaluation.Histopathological examination confirmed mesenteric AF with negative surgical margins.During the 6-month follow-up,computed tomography angiography(CTA)showed unobstructed blood flow of the SMA,and no tumor recurrence or metastasis was observed.The literature review included this case and 9 reported cases,totaling10 cases:7 males and3 females,with an average age of 43.2 years(range,27 to 69 years).Tumor locations included the small intestinal mesentery(5 cases),ileocecal mesentery(1 case),colorectal mesentery(2 cases),and unknown mesenteric sites(2 cases).All patients received surgical treat-ment.One patient died on the first postoperative day,the prognosis of 2 patients was unknown,and the remaining patients had generally favorable outcomes during follow-up.Conclusions AF is a rare borderline soft tissue tumor.The intra-abdominal subtype is particularly uncommon and poses substantial challenges in diagnosis and management.Individualized treatment strategies should be formulated based on tumor type and size.For mesen-teric AF involving the SMA,organ-sparing radical resection combined with vascular reconstruction is a safe and feasible approach.

肖竞楠;陈国栋;唐卫平

南华大学附属第一医院肝胆胰外科,湖南 衡阳 421001南华大学附属第一医院肝胆胰外科,湖南 衡阳 421001南华大学附属第一医院肝胆胰外科,湖南 衡阳 421001

肠系膜侵袭性纤维瘤病肠系膜上动脉包绕肠系膜上动脉重建

mesenteric aggressive fibromatosissuperior mesenteric artery encasementsuperior mesenteric artery reconstruction

《中国现代手术学杂志》 2026 (2)

92-99,8

国家自然科学基金项目(82560612)湖南省卫生健康委科研立项课题(202202054239)

10.16260/j.cnki.1009-2188.2026.02.002

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