Desmoid-type fibromatosis (DF)

  • 文章类型: Journal Article
    纤维瘤病(DF)是一种局部侵袭性肿瘤,其特征是肿瘤细胞周围浸润和远处转移障碍。本系统评价了热消融治疗DF肿瘤的疗效和安全性。
    使用PubMed进行了文献检索,WebofScience,科克伦图书馆,和Embase从2000年1月1日至2022年11月12日。系统审查和荟萃分析(PRISMA)的首选报告项目用于指导文献选择。纳入标准如下:(I)患者病理诊断为侵袭性纤维瘤病,(二)患者接受热消融治疗,(III)关注治疗效果和安全性。同时,排除标准如下:(I)增生性瘢痕患者队列,加德纳纤维瘤,或结节性筋膜炎;(二)会议摘要,reviews,病例报告,给编辑的信,注释,或社论;(III)患者人数<5;(IV)体外或动物实验;(V)非英语文章。使用具有随机效应模型的逆方差方法来获得合并数据。进行亚组分析以确定治疗因素。进行Egger测试以评估发表偏倚的风险。
    在文献选择之后,在23项研究中鉴定出694例DF肿瘤。就模态而言,13项研究使用冷冻消融,9项研究使用高强度聚焦超声(HIFU),1项研究采用微波消融(MWA)。合并症状缓解率为90%[95%置信区间(CI):80-97%],HIFU为100%(95%CI:85-100%),冷冻消融为87%(95%CI:74-97%),MWA为89%(95%CI)。合并的主要并发症发生率为3%(95%CI:1-7%),每种模式的HIFU=2%(95%CI:0-6%),冷冻消融=4%(95%CI:1-8%),MWA=11%,超声=6%(95%CI:1-13%),计算机断层扫描(CT)=2%(95%CI:0-7%),磁共振成像(MRI)=3%(95%CI:0-14%)。合并非灌注体积率(NPVR)为76%(95%CI:71-81%),每种模式的HIFU=77%(95%CI:71-85%),冷冻消融=74%(95%CI:69-79%),超声=75%(95%CI:67-83%),CT=76%(95%CI:67-87%),MRI=78%(95%CI:70-87%)。合并的局部控制率为88%(95%CI:79-94%),每种方式的控制率如下:HIFU=99%(95%CI:96-100%),冷冻消融=80%(95%CI:68-90%),MWA=78%。在主要并发症发生率(P=0.77)和NPVR之间的成像引导模式(P=0.40)差异无统计学意义。消融技术之间的症状缓解率(P=0.32)和主要并发症发生率(P=0.61)也没有差异;然而,消融技术之间的局部控制率差异有统计学意义(P=0.01)。
    成像引导热消融治疗有助于症状缓解,持续时间超过6个月,DF肿瘤的主要并发症发生率低。
    UNASSIGNED: Desmoid-type fibromatosis (DF) is a locally aggressive tumor characterized by peripheral infiltration of neoplastic cells and remote metastasis disability. This systematic review examined the efficacy and safety of thermal ablative therapy for DF tumors.
    UNASSIGNED: A literature search was conducted using PubMed, Web of Science, Cochrane Library, and Embase from January 1, 2000, to November 12, 2022. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement was used to guide literature selection. The inclusion criteria were the following: (I) the patients were diagnosed with aggressive fibromatosis pathologically, (II) the patients were treated by thermal ablations, and (III) a focus on treatment efficacy and safety. Meanwhile, the exclusion criteria were the following: (I) cohorts of patients with hypertrophic scar, Gardner fibroma, or nodular fasciitis; (II) conference abstracts, reviews, case reports, letters to editors, comments, or editorials; (III) number of patients <5; (IV) in vitro or animal experiments; and (V) non-English language articles. The inverse variance method with a random effects model was used to obtain the pooled data. Subgroup analyses were performed to identify treatment factors. Egger test was conducted to assess the risk of publication bias.
    UNASSIGNED: After literature selection, 694 DF tumors were identified in 23 studies. In terms of modality, 13 studies used cryoablation, 9 studies used high-intensity focused ultrasound (HIFU), and 1 study used microwave ablation (MWA). The pooled symptom relief rate was 90% [95% confidence interval (CI): 80-97%], with that for HIFU being 100% (95% CI: 85-100%), that for cryoablation being 87% (95% CI: 74-97%), and that MWA being 89% (95% CI). The pooled major complication rate was 3% (95% CI: 1-7%), and that for each modality was as follows: HIFU =2% (95% CI: 0-6%), cryoablation =4% (95% CI: 1-8%), MWA =11%, ultrasound =6% (95% CI: 1-13%), computed tomography (CT) =2% (95% CI: 0-7%), and magnetic resonance imaging (MRI) =3% (95% CI: 0-14%). The pooled nonperfused volume rate (NPVR) was 76% (95% CI: 71-81%), and that for each modality was as follows: HIFU =77% (95% CI: 71-85%), cryoablation =74% (95% CI: 69-79%), ultrasound =75% (95% CI: 67-83%), CT =76% (95% CI: 67-87%), and MRI =78% (95% CI: 70-87%). The pooled local control rate was 88% (95% CI: 79-94%) and that for each modality was as follows: HIFU =99% (95% CI: 96-100%), cryoablation =80% (95% CI: 68-90%), and MWA =78%. The differences in major complication rate (P=0.77) and NPVR between imaging-guided modalities (P=0.40) were not significant, nor were the differences in symptom relief rate (P=0.32) and major complication rate (P=0.61) between ablative techniques; however, the differences in local control rate (P=0.01) were significant between ablative techniques.
    UNASSIGNED: Imaging-guided thermal ablative therapies contribute to symptom relief with a duration of more than 6 months and a low major complication rate of DF tumors.
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  • 文章类型: Case Reports
    未经证实:乳腺纤维瘤病(BDF)是一种罕见的间叶性肿瘤,仅占乳腺实性肿瘤的0.2%。它被归类为中间肿瘤,因为它是局部侵袭性的,但没有转移潜力。它的诊断通常很困难,因为它与乳腺癌共享许多临床和放射学方面,因此依赖于解剖病理学分析,可以通过遗传分析进行补充。BDF的治疗在过去几年中已经有了相当大的发展。虽然手术是2000年代之前管理的基石,最近的数据显示了从诊断时开始主动监测(AS)的价值.的确,经过两年的AS,该疾病的无进展生存期(PFS)与手术相同或优于手术.此外,在30%的AS患者中观察到自发消退.在疾病进展的情况下,手术可以根据具体情况考虑,以及系统治疗。
    UNASSIGNED:我们介绍了一例双侧BDF的病例,该病例影响了一名20岁的女性,其第一个建议的治疗方法是双侧乳房切除术和重建术。在第二意见之后,该决定进行了修订,并启动了AS。AS发病后将近3年,肿瘤已显示出持续消退。
    UNASSIGNED:该病例表明需要在间充质肿瘤的治疗方面有经验,以避免因残害手术而导致的过度治疗,从而促进复发。此外,根据我们的知识,迄今为止,很少有双边BDF病例发表。因此,我们似乎有必要报告这个罕见的病例,这支持了AS对DF的兴趣,正如最近由Desmoid肿瘤工作组指南所建议的那样。
    UNASSIGNED: Breast desmoid-type fibromatosis (BDF) is a rare mesenchymal tumor accounting for only 0.2% of solid breast tumors. It is classified as an intermediate tumor because it is locally aggressive but has no metastatic potential. Its diagnosis is often difficult because it shares many clinical and radiologic aspects with breast carcinomas and therefore relies on anatomopathological analysis which may be supplemented by genetic analysis. The treatment of BDF has considerably evolved in the past years. While surgery was the cornerstone of the management prior to the 2000s, recent data have shown the value of active surveillance (AS) from the time of diagnosis. Indeed, after 2 years of AS, the progression-free survival (PFS) of the disease is identical or superior to surgery. Moreover, spontaneous regression has been observed in 30% of patients undergoing AS. In case of disease progression, surgery can be considered on a case-by-case basis, as well as systemic treatments.
    UNASSIGNED: We present a case of bilateral BDF affecting a 20-year-old woman for whom the first suggested treatment was bilateral mastectomy with reconstruction. After a second opinion, the decision was revised and AS was initiated. Almost 3 years after the onset of AS, tumors have shown a continuous regression.
    UNASSIGNED: This case demonstrates the need for experience in the management of mesenchymal tumors to avoid overtreatment by mutilating surgeries which promote recurrence. Moreover, to our knowledge, very few cases of bilateral BDF have been published to date. It thus seemed relevant for us to report this rare case which supports the interest of AS for DF, as recently advised by the Desmoid Tumor Working Group guidelines.
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