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与韧带样型纤维瘤病疾病稳定相关的因素。

Factors Associated with Disease Stabilization of Desmoid-Type Fibromatosis.

机构信息

Musculoskeletal Tumor Center, Seoul National University Cancer Hospital, Seoul, Korea.

East Avenue Medical Center, Quezon City, Philippines.

出版信息

Clin Orthop Surg. 2020 Mar;12(1):113-119. doi: 10.4055/cios.2020.12.1.113. Epub 2020 Feb 13.

DOI:10.4055/cios.2020.12.1.113
PMID:32117547
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7031434/
Abstract

BACKGROUND

Spontaneous disease stabilization of desmoid-type fibromatosis (DF) has been demonstrated in many reports, and the watchful waiting approach without any frontline treatment is becoming popular as an initial management strategy. In this study, we aimed to assess the disease stabilization rate and identify predictive factors for disease stabilization of DF in patients with conservative treatment.

METHODS

We reviewed 76 patients with sporadic extra-abdominal DF who were managed with frontline conservative treatment in our institute. The minimum follow-up was 12 months. Stabilization was defined as radiological evidence of no change or continuous decrease in size of the tumor for six months or more. The primary endpoint was stabilization of DF. Possible patient-, disease-, and treatment-related factors predictive of disease stabilization were analyzed with multivariate analysis.

RESULTS

At final follow-up, 54 of the 76 tumors (71%) were stable, and mean time to stabilization was 30.4 months (range, 7 to 112 months). On Kaplan-Meier survival analysis, the spontaneous stabilization rate was 25.4% at one year, 52.7% at two years, and 70.9% at three years. The mean time to spontaneous stabilization was longer in patients with ≤ 40 years of age ( = 0.022) or recurrence ( = 0.041). On multivariate analysis with the Cox proportional hazard method, recurrence (hazard ratio [HR], 1.79; = 0.041) and younger age (HR, 2.04; = 0.022) were identified as independent prognostic factors for longer time to disease stabilization.

CONCLUSIONS

Frontline conservative treatment seems to be the optimal treatment for most patients with DF. Younger patients or those with recurrence may require longer time to spontaneous disease stabilization.

摘要

背景

许多报道已经证实,硬纤维瘤(desmoid-type fibromatosis,DF)会自发稳定,观察等待而不进行任何一线治疗的方法正成为初始管理策略。在本研究中,我们旨在评估保守治疗患者中 DF 稳定的发生率,并确定其稳定的预测因素。

方法

我们回顾了在我们医院接受一线保守治疗的 76 例散发性腹外型 DF 患者。随访时间至少为 12 个月。稳定定义为肿瘤的影像学证据显示至少连续 6 个月无变化或持续缩小。主要终点为 DF 稳定。采用多因素分析评估可能与患者、疾病和治疗相关的预测 DF 稳定的因素。

结果

在最终随访时,76 个肿瘤中有 54 个(71%)稳定,平均稳定时间为 30.4 个月(范围,7 至 112 个月)。Kaplan-Meier 生存分析显示,1 年、2 年和 3 年的自发稳定率分别为 25.4%、52.7%和 70.9%。年龄≤40 岁( = 0.022)或复发的患者自发稳定时间更长( = 0.041)。采用 Cox 比例风险方法进行多因素分析,发现复发(风险比 [HR],1.79; = 0.041)和年轻(HR,2.04; = 0.022)是疾病稳定时间更长的独立预后因素。

结论

一线保守治疗似乎是大多数 DF 患者的最佳治疗方法。年轻患者或复发患者可能需要更长时间才能自发稳定疾病。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/05ea/7031434/f3115477e5d1/cios-12-113-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/05ea/7031434/f3115477e5d1/cios-12-113-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/05ea/7031434/f3115477e5d1/cios-12-113-g001.jpg

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