Risk factors of local recurrence after surgery in extraabdominal desmoid-type fibromatosis: A multicenter study in Japan

Risk factors of local recurrence after surgery in extraabdominal desmoid-type fibromatosis: A multicenter study in Japan
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DOI:
10.1111/cas.14528
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发表时间:
2020-07-11
期刊:
影响因子:
5.7
通讯作者:
Koike, Hiroshi
Koike, Hiroshi
中科院分区:
医学2区
文献类型:
--
作者:
Nishida, Yoshihiro;Hamada, Shunsuke;Koike, Hiroshi

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本研究旨在阐明包括CTNNB1基因突变状态在内的少见疾病韧带样型纤维瘤病术后局部复发的危险因素。它被设计为一个多机构联合研究项目,日本的7个主要中心参与其中。7个主要骨和软组织肿瘤医学中心的委员会成员组成了这个研究小组,以制定临床护理指南。从7个机构收集的196例标本和病历中,88例手术治疗的病例进行了临床病理预后因素分析,包括CTNNB1突变状态。排除R2(n=3),5年无复发生存率为52.9%。无一例在术前或术后接受放射治疗。单因素分析显示,肢体位置(P<.001)和较大尺寸(8 cm或以上,P=.036)是LRFS的显著不良危险因素。多因素分析显示,除肿瘤复发(P=.041)、S45F突变(P=.028)和R1手术切缘(P=.039)外,肢体位置(P<.001)是一个显著的不利因素。术前药物治疗,包括非类固醇抗炎药,并不能降低局部复发的发生率(P=0.199)。这是第一次分析与手术治疗结果相关的因素,包括CTNNB1突变状态,来自亚洲国家的病例数量相对较多。肿瘤部位被发现是影响局部复发的最有影响的预后因素,与欧洲和北美的结果相似。建立更灵敏的CTNNB1突变检测方法(S)是今后研究的重点。
This study was undertaken to clarify the risk factors, including the mutation status ofCTNNB1, for the local recurrence after surgery of the rare disease desmoid-type fibromatosis. It was designed as a multiinstitutional joint research project with 7 major centers in Japan participating. The committee members of 7 major medical centers specializing in bone and soft tissue tumors formed this study group to develop clinical care guidelines. Of 196 cases with specimens and medical records collected from the 7 institutions, 88 surgically treated ones were analyzed regarding clinicopathologic prognostic factors includingCTNNB1mutation status. Excluding R2 cases (n = 3), 5-year local recurrence-free survival (LRFS) was 52.9%. No case had received pre- or postoperative radiotherapy. Univariate analysis revealed that extremity location (P < .001) and larger size (8 cm or more,P = .036) were significant adverse risk factors for LRFS. Multivariate analysis indicated that extremity location (P < .001) was a significantly adverse factor in addition to recurrent tumor (P = .041), S45F mutation (P = .028), and R1 surgical margin (P = .039). Preoperative drug treatment, including nonsteroidal antiinflammatory drugs, did not reduce the incidence of local recurrence (P = .199). This is the first study to analyze the factors correlating with outcomes of surgical treatment, includingCTNNB1mutation status, in a relatively large number of cases from an Asian country. Tumor location was found to be the most influential prognostic factor for local recurrence, similar to the results from Europe and North America. The development of more sensitive method(s) for determination ofCTNNB1mutation is a priority for future study.