Department of Orthopedics and Trauma, Medical University of Graz, Auenbruggerplatz 6, 8036 Graz, Austria.
Institute of Pathology, Medical University of Graz, Neue Stiftingtalstraße 6, 8010 Graz, Austria.
Orthop Traumatol Surg Res. 2020 Oct;106(6):1059-1065. doi: 10.1016/j.otsr.2020.04.017. Epub 2020 Aug 7.
Myxofibrosarcoma (MFS) is one of the most common sarcoma subtype in elderly patients. They are reported to recur locally independently of the tumour grade in 30-40% of cases and metastases are reported to develop in high-grade tumours in 20-35% cases. As MFS is a rare diagnosis, data investigating specific survival and independent risk factors are lacking and have mostly been limited to single orthopaedic oncology centre studies so far. Thus we set up a pathology-based retrospective study and analyzed all MFS diagnosed in our institution with the following aims: (1) analysis of independent risk factors for overall survival, disease specific survival, local recurrence-free survival and distant metastasis free survival following resection of MFS; (2) analysis of resection margin status.
High-grade MFS have a low survival distant metastasis free survival and local recurrence free survival is dependent on surgical margin status.
We retrospectively analysed 109 patients (median 66 years [range, 21-96]) diagnosed with MFS and a median follow-up of 42 months at one centre between 1990 and 2014. Tumor-associated survival, including competing risk analysis, and prognostic factors for local recurrence, metastatic disease and death from disease were investigated and included in a multivariate analysis.
Overall survival was 79% [95%CI: 71.9-87.5] at 3 years and 76% [95%CI: 67.4-84.6] at 5 years. Disease specific survival was 85% [95%CI: 78.4-92.2] at 3 years and 80% [95%CI: 72.2-88.2] at 5 years. There were local recurrences in 11/109 patients (10%). Local recurrence free survival (LRFS) was 95% [95%CI: 92.0-99.8] at 3 and 88% [95%CI: 84.3-96.4] at 5 years. Metastatic disease (n=25; 23%) occurred after a median follow-up of 10 months. Distant metastasis free survival was 78% [95%CI: 69.9-85.9] at 3 and 77% [95%CI: 68.4-84.8] at 5 years. R1 status at primary resection was an independent risk factor for decreased Local Recurrence-free survival (OR: 8.5, 95%CI: 1.59-49.79 [p=0.01]). Grading was an independent risk factor for decreased Disease specific survival (OR 13.4, 95%CI: 1.65-1734.84 [p=0.01]) and Distant metastasis free survival (OR 16.2, 95%CI: 2.0-2110.5 [p=0.004]). Primary resection achieved R0 margins in 63 (58%) of 109 patients. Margins were adequate significantly more often (p<0.001) in patients treated primarily at a sarcoma centre (R0=58/68, 85%) than in those treated primarily at non-sarcoma centres (R0= 5/41, 12%), whereby the latter significantly more often treated superficial tumours (p=0.001) with a size of less than 5cm (p<0.001).
Patients with high-grade MFS had a poorer prognosis with respect to Disease specific survival/Distant metastasis free survival than low-grade MFS. Local recurrence did not significantly affect disease specific survival.
IV.
黏液纤维肉瘤（MFS）是老年人中最常见的肉瘤亚型之一。据报道，在 30-40%的病例中，肿瘤分级独立于局部复发，在 20-35%的高分级肿瘤中转移。由于 MFS 是一种罕见的诊断，缺乏调查特定生存率和独立危险因素的数据，并且迄今为止主要局限于单个骨科肿瘤中心的研究。因此，我们建立了一项基于病理学的回顾性研究，并分析了我们机构诊断的所有 MFS，目的如下：（1）分析 MFS 切除后总生存率、疾病特异性生存率、局部无复发生存率和远处无转移生存率的独立危险因素；（2）分析切除边缘状态。
高级 MFS 的远处无转移生存率和局部无复发生存率较低，手术边缘状态取决于局部无复发生存率。
我们回顾性分析了 1990 年至 2014 年期间在一个中心诊断为 MFS 的 109 例患者（中位年龄 66 岁[范围，21-96]），中位随访时间为 42 个月。调查了与肿瘤相关的生存率，包括竞争风险分析，以及局部复发、转移性疾病和疾病死亡的预后因素，并纳入多变量分析。
3 年总生存率为 79%[95%CI：71.9-87.5]，5 年总生存率为 76%[95%CI：67.4-84.6]。3 年疾病特异性生存率为 85%[95%CI：78.4-92.2]，5 年疾病特异性生存率为 80%[95%CI：72.2-88.2]。109 例患者中有 11 例（10%）出现局部复发。3 年局部无复发生存率为 95%[95%CI：92.0-99.8]，5 年局部无复发生存率为 88%[95%CI：84.3-96.4]。中位随访 10 个月后发生转移性疾病（n=25；23%）。3 年远处无转移生存率为 78%[95%CI：69.9-85.9]，5 年远处无转移生存率为 77%[95%CI：68.4-84.8]。R1 状态为原发性切除的是局部无复发生存率降低的独立危险因素（OR：8.5，95%CI：1.59-49.79[P=0.01]）。分级是疾病特异性生存率降低的独立危险因素（OR 13.4，95%CI：1.65-1734.84[P=0.01]）和远处无转移生存率降低的独立危险因素（OR 16.2，95%CI：2.0-2110.5[P=0.004]）。109 例患者中，63 例（58%）原发性切除达到 R0 切缘。在主要在肉瘤中心治疗的患者中，切缘充足的比例显著更高（p<0.001）（R0=58/68，85%），而在主要在非肉瘤中心治疗的患者中，切缘充足的比例显著更低（R0=5/41，12%），后者更常治疗大小小于 5cm 的浅表肿瘤（p=0.001）。
高级 MFS 患者的疾病特异性生存率/远处无转移生存率比低级别 MFS 患者差。局部复发对疾病特异性生存率无显著影响。
IV。
