侵袭性纤维瘤病48例临床特点及诊治分析

Clinical characteristics, diagnosis and treatment of aggressive fibromatosis: An analysis of 48 cases

目的探讨侵袭性纤维瘤病(AF)的临床特点、诊断、治疗及预后。方法回顾性分析大连医科大学附属第一医院2002年3月至2017年3月收治的48例AF病人的临床资料,对其临床特点、免疫表型、治疗方法及预后因素进行分析。结果 48例病人中,男性21例,女性27例;年龄1~76岁,其中31~40岁病例最多(13例)。腹外型34例(70.8%),腹壁型9例(18.8%),腹腔内和腹内型(10.4%)。均行外科手术治疗,免疫组化β-连环蛋白(β-catenin)、波形蛋白(vimentin)阳性率较高,分别为100.0%、87.5%,术后完整随访40例(83.3%),4例病人术后复发。结论 AF病好发于年轻女性,高发年龄为31~40岁,除肿瘤本身的生物学行为及常规影像特点外,免疫组化β-连环蛋白、波形蛋白阳性为AF的特征性表现,手术切除仍为最重要的治疗方式。术前应积极明确诊断,应充分评估完整切除的可能性与手术创伤的风险,将手术引起的器官和功能障碍与肿瘤的预期行为和其他治疗的潜在疗效进行权衡。无论瘤体大小,术中均应送检标本切缘,以确保R0切除,减少复发率。

Objective To investigate the clinical characteristics, diagnosis, treatment and prognosis of aggressive fibromatosis （AF）. Methods The clinical data of 48 patients of AF admitted in the Department of Abdominal Emergency Surgery, the First Affiliated Hospital of Dalian Medical University from March,2002 to March,2017 were analyzed retrospectively. The clinical features, immunophenotype, treatment and prognosis of patients of AF were analyzed retrospectively. Results All the cases included 21 males and 27 females, aged 1 to 76 years, with the most cases （13 cases） aged 31-40 years. There were 34 cases （70.8%） of extra-abdominal AF, 9 cases （18.8%） of abdominal wall AF, 5 cases （10.4%） of intra-abdominal AF. The positive rate of β-catenin and vimentin were 100.0% and 87.5% respectively. Forty patients were followed-up, of which 4 recurred after operation.Conclusion AF occurs in young women, high incidence of 31-40 years old, in addition to the biological behavior of the tumor itself and the characteristics of conventional imaging, immunohistochemistry 13-catenin, vimentin positive are the performance of AF. Surgical resection is the most important treatment. Preoperative diagnosis should be made and the risk of complete resection should be adequately assessed and the risk of surgical trauma should be used to weigh the organ and dysfunction caused by surgery with the expected behavior of the tumor and the potential efficacy of other treatments. The margins should be sent to the pathology to ensure the R0 resection, reduce the recurrence rate regardless of tumor size.