Extra-abdominal primary fibromatosis:: Aggressive management could be avoided in a subgroup of patients
Extra-abdominal primary fibromatosis:: Aggressive management could be avoided in a subgroup of patients
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DOI：
10.1016/j.ejso.2007.06.006
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发表时间：
2008-04-01
期刊：
影响因子：
3.8
通讯作者：
Le Pechoux, C.
中科院分区：
文献类型：
--
作者：
Bonvalot, S.;Eldweny, H.;Le Pechoux, C.
目的：评价手术作为一线治疗对原发性侵袭性纤维瘤病无事件生存期（EFS）的影响。患者和方法：治疗分为：手术加放疗或不加放疗，非手术策略单独全身治疗或观望政策。89例患者行原发肿瘤切除术，13例术后行放射治疗。23人没有接受手术，但接受了系统治疗或观察等待政策。结果：中位随访时间为76个月。总体3年EFS为49%。在单变量分析中，显微镜下完全手术的患者与非手术组患者的结果相似（3年EFS分别为65%和68%）。性别、年龄、肿瘤大小、治疗期和治疗策略（手术与非手术）无统计学意义。根据切缘和肿瘤部位切除的质量是唯一的预后因素。肿瘤部位与手术质量有显著相关性（p = 0.0002）。结论：一部分腹外纤维瘤病患者可以采用非侵袭性策略进行治疗，因为2/3的非手术患者生长停止。当最终需要手术时，应以达到负切缘为目标进行手术。(C) 2007 Elsevier Ltd.版权所有。
Objective: To evaluate the impact of surgery as first-line treatment on event-free survival (EFS) of primary aggressive fibromatosis.Patients and methods: Treatments were categorized into: surgery with or without radiotherapy and nonsurgical strategies with systemic treatment alone or wait and see policy. Eighty-nine patients had initial resection of their primary tumour followed by postoperative radiotherapy in 13 cases. Twenty-three did not undergo surgery but received systemic treatment or watch and wait policy.Results: Median follow-up was 76 months. Overall 3 years EFS was 49%. In the univariate analysis, patients with microscopically complete surgery had a similar outcome to patients in the no-surgery group (3 years EFS of 65% and 68%, respectively). Gender, age, tumour size, treatment period and strategy (surgery versus no-surgery) were not statistically significant. Quality of resection according to margins and the tumour site were the only prognostic factors. There was a significant correlation between tumour site and quality of surgery (p = 0.0002).Conclusions: A subset of patients with extra-abdominal fibromatosis could be managed with a nonaggressive policy, as growth arrest concerned 2/3 of nonoperated patients. When surgery is finally necessary, it should be performed with the aim of achieving negative margins. (C) 2007 Elsevier Ltd. All rights reserved.