背景:纤维瘤是良性纤维瘤,起因于肌膜膜结构内肌成纤维细胞增殖失调。它们可以偶尔发生,但更常见的是与遗传综合征有关,例如家族性腺瘤性息肉病(Sakorafas等。在SurgOncol16(2):131-142,2007)(FAP)。任一Wnt的突变,β-连环蛋白或APC基因是这些肿瘤发展的“关键”触发因素(Howard和Pollock在OncolTher4(1):57-72,2016)。经典的,这些肿瘤不会转移;然而,由于它们的浸润模式和/或局部侵袭,它们与显著的发病率和死亡率相关.历史上,手术切除是治疗的基石。关于腹部硬纤维瘤手术治疗成功后的结果的数据仍然很少。复发和发病率。
目的:本综述的目的是评估目前腹部硬纤维瘤手术治疗成功的证据,复发和发病率。
方法:对PubMed中的文章进行系统搜索,EMBASE和Cochrane图书馆数据库是根据2000年1月至2020年11月期间系统审查和荟萃分析指南的首选报告项目进行的。
结果:纳入了23项研究,其中,749例患者进行了手术切除(原发性696例,复发性纤维瘤53例),243例患者(18.8%)接受了医学管理,353例患者(27.3%)接受了监测。中位随访时间为51.4个月(范围1-372)。749例切除手术中有696例(92.9%)进行了原发性韧带样切除术,其余53例(7.1%)因复发而接受切除术。一百零二例手术管理的患者(19%)出现(重新)复发,肠系膜受累是最常见的复发部位(55%)。当比较手术后复发与药物治疗后的进展时,手术有更好结果的趋势,25%的手术患者复发,50.5%的患者药物治疗进展[OR0.40(95%CI0.06-2.70),p=0.35]。手术后的主要发病率为4.4%(n=33),切除后30天内的死亡率为2%(n=14)。
结论:纤维瘤的管理具有相当大的异质性。在可以获得阴性切缘的高度选择性病例中,手术切除腹部硬纤维瘤仍然是一种有效的治疗选择。主要发病率和/或死亡率低。
BACKGROUND: Desmoid tumours are benign fibromatous tumours arising from dysregulated myofibroblast proliferation within musculoaponeurotic structures. They can occur sporadically but more commonly are associated with genetic syndromes such as familial adenomatous polyposis (Sakorafas et al. in Surg Oncol 16(2):131-142, 2007) (FAP). Mutations in either the Wnt, β-catenin or APC genes are \'key\' triggers for the development of these tumours (Howard and Pollock in Oncol Ther 4(1):57-72, 2016). Classically, these tumours do not metastasise; however, they are associated with significant morbidity and mortality due to their infiltrative pattern and/or local invasion. Historically, surgical resection was the cornerstone of treatment. There remains paucity of data regarding outcomes following the surgical management of abdominal desmoid tumours in terms of success, recurrence and morbidity.
OBJECTIVE: The aim of this
review was to assess the current evidence for surgical management of abdominal desmoid tumours in terms of success, recurrence and morbidity.
METHODS: A systematic search of articles in PubMed, EMBASE and The Cochrane Library databases was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines for the period from January 2000 to November 2020.
RESULTS: Twenty-three studies were included, of which, 749 patients had surgical resection (696 for primary and 53 for recurrent desmoids), 243 patients (18.8%) were medically managed and 353 patients (27.3%) underwent surveillance. Median follow-up was 51.4 months (range 1-372). Six-hundred and ninety-six of the 749 resections (92.9%) underwent primary desmoid resection, with the remaining 53 (7.1%) undergoing resection for recurrence. One-hundred and two surgically managed patients (19%) developed a (re)recurrence, with mesenteric involvement the commonest site for recurrence (55%). When comparing recurrence post-surgery to progression following medical therapy, there was a trend towards better outcomes with surgery, with 25% of surgical patients having a recurrence versus 50.5% having progression with medical therapy [OR 0.40 (95% CI 0.06-2.70), p = 0.35]. Major morbidity following surgery was 4.4% (n = 33) with 2% (n = 14) mortality within 30 days of resection.
CONCLUSIONS: The management of desmoids has considerable heterogeneity. Surgical resection for abdominal desmoids remains a valid treatment option in highly selective cases where negative margins can be obtained, with low major morbidity and/or mortality.