【摘要】 背景与目的：侵袭性纤维瘤病（AF）是一种罕见的、来源于深部软组织的克隆性肌成纤维细胞增殖性疾病。本研究通过分析总结湖南省内21家医疗中心收治的腹壁AF患者的病例特点以及临床病理特征，了解腹壁AF的认识现状，探讨影响腹壁AF患者复发的相关因素。方法：回顾性收集湖南省内21家医疗中心2010年1月1日—2022年9月30日期间连续收治的148例腹壁AF患者的临床病例资料及随访结果，分析患者的基本特征、治疗方式、术后复发的危险因素及预后。结果：148例腹壁AF患者中，男性15例（10.1%），女性133例（89.9%）；年龄3～72岁，中位年龄为31岁；初发患者142例（95.9%），复发患者6例（4.1%）;71例（48.0%）患者既往有腹部手术史。女性患者中，111例（83.5%）有妊娠史，57例（42.9%）有剖宫产史。148例患者均通过术后病理学检查明确诊断。术前通过细针穿刺获得病理诊断16例（10.8%），其中8例（50.0%）术前病理与术后病理结果一致。100例患者（67.6%）术后行免疫组织化学检查。其中，32例（32.0%）患者Ki-67>5%,56例（56.0%）患者β-catenin (+),42例（42.0%）患者vimentin （+）。148例腹壁AF患者均行腹壁肿瘤根治性切除术，其中72例切除肿瘤后采取直接缝合修复腹壁，72例采用人工网片加强重建腹壁，4例采用肌皮瓣移植或带血管蒂阔筋膜张肌肌皮瓣移植术重建腹壁。127例（85.8%）通过电话及门诊等方式获随访，中位随访时长为3.6 (0.9～12.3)年。随访期间，11例（8.7%）出现复发，其中3例为二次复发。复发型AF和手术切缘阳性是术后复发的危险因素（均P<0.05）。无术后伤口感染、伤口裂开或切口疝发生，无肿瘤转移或死亡发生。结论：腹壁AF绝大多数发生于有妊娠史的年轻女性，肿瘤根治切除术后仍有一定的复发率。外科医生应尽可能确保手术切缘阴性，尤其对复发型AF患者，应建议到有经验的疝和腹壁外科中心制定个体化方案进行专业治疗。更多还原

【Abstract】 Background and Aims: Aggressive fibromatosis(AF) is a rare clonal myofibroblast proliferative disease derived from deep soft tissues. This study aims to analyze and summarize the case characteristics and clinicopathologic features of patients with abdominal wall AF admitted to 21 medical centers in Hunan province, to enhance the current knowledge and investigate the factors associated with the recurrence of abdominal wall AF.Methods: The clinical data and follow-up results of 148 patients with abdominal wall AF recruited consecutively from 21 medical centers in Hunan province between January 1, 2010 and September 30, 2022 were reviewed. The general characteristics, treatment method, risk factors for postoperative recurrence, and prognosis of patients were analyzed.Results: Among 148 patients with abdominal wall AF, 15 cases(10.1%) were males, and 133 cases(89.9%) were females with a median age of 31(3-72) years old; there were 142 cases(95.9%) with primary disease and 6 cases(4.1%) with recurrent disease; 71 cases(48.0%) had a history of abdominal surgery. Among the female patients, 111 cases(83.5%) had a history of pregnancy, and 57 cases(42.9%) had a history of cesarean section. All patients were confirmed with AF by postoperative pathological examination. Pathological diagnosis obtained by fine needle puncture before surgery in 16 patients(10.8%), which in 8 cases(50.0%) were consistent with the postoperative results. Immunohistochemical staining was performed in 100 patients(67.6%). Among them, 32 cases(32.0%) had Ki-67 >5%, 56 cases(56.0%) had β-catenin(+), and 42 patients(42.0%) had vimentin(+). All 148 patients with AF underwent radical resection of abdominal wall tumors. Among them, 72 cases underwent abdominal wall repair by direct suture after tumor resection, 72 cases underwent mesh implant for abdominal wall strengthening, and 4 cases underwent abdominal wall reconstruction with a musculocutaneous flap or pedicled tensor fascia lata musculocutaneous flap. Follow-up was obtained through telephone calls and clinic visits in 127 patients(85.8%), and the median follow-up time was 3.6(0.9-12.3) years. During follow-up, recurrence occurred in 11 patients(11/127, 8.7%), including three re-recurrent cases. Recurrent AF and positive surgical margins were risk factors for postoperative recurrence(both P<0.05). Postoperative wound infection and wound dehiscence or incisional hernia were observed in none of them, and no tumor metastasis or death occurred.Conclusions: Abdominal wall AF predominantly affects young female adults with a pregnancy history. There is a certain recurrence rate after radical resection. Surgeons should strive to achieve negative surgical margins, especially for those with recurrent AF, for whom specialized care with an individualized treatment plan from an experienced hernia and abdominal wall surgery center is recommended.更多还原