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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 features, diagnosis, treatment and prognosis of invasive fibromatosis (AF). Methods The clinical data of 48 AF patients admitted to the First Affiliated Hospital of Dalian Medical University from March 2002 to March 2017 were retrospectively analyzed. The clinical features, immunophenotypes, treatment methods and prognostic factors were analyzed. Results Of the 48 patients, 21 were males and 27 were females, ranging in age from 1 to 76 years, of whom 31 were the most frequent (13 cases). 34 cases (70.8%) had abdominal abnormality, 9 cases (18.8%) had abdominal wall, and (10.4%) intraperitoneally and intra-abdominal. The positive rates of β-catenin and vimentin were 100.0% and 87.5% respectively after surgery. The complete follow-up of 40 cases (83.3%) and 4 cases The patient recurred after surgery. CONCLUSIONS: AF is a common disease in young women with a high incidence of 31- to 40-year-old. In addition to the biological behavior and imaging characteristics of the tumor itself, immunohistochemistry of β-catenin and vimentin are characteristic features of AF. Surgical resection Still the most important treatment. Positive diagnosis should be actively performed before surgery. The possibility of complete resection and the risk of surgical trauma should be adequately assessed. The organ and dysfunction caused by surgery should be weighed against the expected behavior of the tumor and the potential efficacy of other treatments. No matter the size of the tumor, surgery should be sent to check the margin of the specimen to ensure R0 resection and reduce the recurrence rate.