腹壁硬纤维瘤诊疗进展  

Advancements in the diagnosis and treatment of abdominal wall desmoid tumors

作　　者：刘柏岐 黄耿文[1] LIU Bai-qi;HUANG Geng-wen(Division of Hernia and Abdominal Wall Surgery,Department of General Surgery,Xiangya Hospital,Central South University,Changsha 410008,China)

机构地区：[1]中南大学湘雅医院普外科疝和腹壁外科中心,湖南长沙410008

出　　处：《中国实用外科杂志》2024年第3期349-355,共7页Chinese Journal of Practical Surgery

基　　金：湖南省自然科学基金资助项目(No.2023JJ30885)。

摘　　要：腹壁硬纤维瘤(DT)是一类发生在腹壁肌层的侵袭性纤维瘤,其发病率较低,临床了解较少。该肿瘤具有局部浸润和复发率高的特点,且病程难以预测。目前对于该疾病的诊断和治疗的认识正在不断完善和加深,治疗策略正不断优化。常用影像学诊断方式包括超声、CT和MRI等。DT的影像学表现具有一定特点,需要与炎性病变或软组织恶性肿瘤相鉴别,最终诊断需要通过活检及组织病理学进行确认。主动监测(AS)作为目前一线治疗策略,对于生长缓慢、无症状或手术风险高的病人,提供了一种避免过度治疗的有效途径。对于需要治疗干预的腹壁DT,首选手术治疗。对于术后切缘阳性或不能手术的腹壁DT,可选择药物治疗或放疗疗法。此外,所有腹壁DT病人应由肉瘤多学科团队进行全面评估和处理,并进行定期的随访和影像学检查,评估治疗效果和复发情况,根据需要调整治疗方案。对于妊娠期DT病人,AS仍为首选治疗方法。对于进展较快的DT病人,孕期积极手术治疗是安全和有效的。但抗肿瘤药物可能会导致卵巢功能障碍相关等不良事件,进而影响育龄女性的生育能力。因此,应在治疗前充分了解可能的生育风险,并考虑向生殖门诊进行咨询。尽管近年来对DT的理解有所进步,但对腹壁DT的诊疗仍无统一标准,需根据个体情况定制治疗计划。Abdominal desmoid tumors(DT)are a type of invasive fibroma that occur in the abdominal wall muscle layer.With a low incidence rate and limited understanding clinically,these tumors are characterized by local infiltration,high recurrence rate,and an unpredictable disease course.The understanding and treatment of this disease are constantly improving and strategies are being optimized.Diagnostic methods include ultrasound,CT,and MRI.The specific imaging manifestations of DT require differentiation from inflammatory diseases or malignant soft tissue tumors.A final diagnosis is confirmed through biopsy and histopathology.Active surveillance(AS)is a current first-line treatment strategy.For patients with slow growth,no symptoms,or high surgical risk,AS is an effective way to avoid overtreatment.For abdominal DT that requires treatment intervention,surgical treatment is the first choice.Positive-margin postoperative or inoperable abdominal DT may be treated with drug therapy or radiotherapy.Furthermore,all abdominal DT patients should be fully evaluated and treated by a sarcoma multidisciplinary team,and undergo regular follow-up and imaging examinations.This will help evaluate treatment effects and recurrence,adjusting treatment plans as necessary.For pregnant DT patients,AS remains the first choice treatment.For patients with fast-growing DT,aggressive surgical treatment during pregnancy is safe and effective.However,antitumor drugs can cause adverse events related to ovarian dysfunction,potentially affecting the fertility of women of childbearing age.Therefore,potential fertility risks should be fully understood before treatment,and consultation with the reproductive clinic is recommended.Even though understanding of DT has improved in recent years,there is still no uniform standard for diagnosing and treating abdominal DT.Individualized treatment plans should be customized according to each case.

关 键 词：腹壁硬纤维瘤 主动监测 靶向药物 

分 类 号：R6[医药卫生—外科学]