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目的探讨韧带样型纤维瘤病(DTFT)的诊断和治疗方法。方法回顾性总结收治的DTFT11例患者的临床资料。肿瘤位于腹壁和盆腔4例,腹腔内2例,腹部以外5例。病程平均为5.7年(1个月至41年)。术前均误诊。2例行局部切除,其余9例均行扩大切除术;3例术后行放射治疗。结果11例术后病理均诊断为DTFT,其中波形蛋白阳性10例,阴性1例。术后随访1～10年(平均4.2年),复发4例,平均复发时间2.2年(8个月至5年)。11例均健康生存,未见有远处转移。结论DTFT在临床上易与其他软组织肿瘤相混淆;诊断主要依据病理报告;治疗DTFT最主要的方法是扩大的手术切除。该病预后较好,复发者可再次手术。单独使用放疗可以作为无法切除肿块的治疗方式。
Objective To investigate the diagnosis and treatment of ligament-like fibromatosis (DTFT). Methods The clinical data of 11 patients with DTFT were retrospectively reviewed. The tumor was located in the abdominal wall and pelvic in 4 cases, intraperitoneal in 2 cases, 5 cases outside the abdomen. The average course of disease was 5.7 years (1 month to 41 years). Preoperative misdiagnosis. 2 cases of local excision, the remaining 9 cases underwent expansive resection; 3 cases underwent radiotherapy. Results All 11 cases were diagnosed as DTFT by pathology, including 10 cases of vimentin positive and 1 case negative. The patients were followed up for 1 to 10 years (mean 4.2 years), and 4 cases were relapsed. The average recurrence time was 2.2 years (ranged from 8 months to 5 years). All 11 cases survived without distant metastasis. Conclusion DTFT is easily confused with other soft tissue tumors in clinic. The diagnosis is mainly based on the pathological reports. The most important method for the treatment of DTFT is extended surgical resection. The prognosis of the disease is better, relapse surgery can be repeated. Radiotherapy alone can be used as a treatment of unresectable lumps.