General and Thoracic Surgery, Istituto Ortopedico Rizzoli, Via Pupilli 1, 40136, Bologna, Italy.
Int Orthop. 2013 Dec;37(12):2429-36. doi: 10.1007/s00264-013-2048-5. Epub 2013 Sep 6.
Myxoinflammatory fibroblastic sarcoma (MIFS) is a rare soft tissue tumour first identified at the end of the 1990s. This study presents our experience and literature reviews focusing on risk of recurrence.
Rizzoli Orthopaedic Institute database and literature were searched for patients with MIFS observed from 1997 to 2012. Data were analysed in a new database.
Five patients underwent surgery at our institute, and 133 cases were retrieved from the literature. Not all clinicopathological data were available: 76/138 were men (55%), median age was 45 [interquartile range (IQR) 34-56] years, median tumour size was three (IQR two to five) centimetres. Common sites of occurrence were hand (24%), fingers (23%) and foot (20%). Pain was present at diagnosis in 14/82 patients (17%), with a median duration of seven (IQR three to 12) months. Surgery was performed for a suspected benign tumour in 88 patients (74%). Resection was incomplete in 45/71 cases (63%); re-excision was performed in 32/45 (71%). At a median follow-up of 26 months, 26/118 patients (22%) developed recurrent disease; median time to recurrence was 15 months (IQR seven to 26). Actuarial relapse-free survival (RFS) at one, three and five years was 93%, 72% and 67%, respectively. At univariate analysis, only symptom duration of six months or less was significantly associated with a worse RFS (p = 0.046). Metastatic disease to lymph nodes and/or lungs was observed in four patients (3%).
Clinicopathological findings confirm the low-grade nature of MIFS. However, local recurrence occurs, and patients may be affected by aggressive forms with a potential for distant metastases. Follow-up is strongly advised.
黏液炎症性纤维母细胞肉瘤（MIFS）是一种罕见的软组织肿瘤，于 20 世纪 90 年代末首次被确定。本研究介绍了我们的经验并对复发风险进行了文献回顾。
检索了里佐利骨科研究所数据库和文献中 1997 年至 2012 年期间观察到的 MIFS 患者的数据。对这些数据在新的数据库中进行了分析。
我们研究所的 5 名患者接受了手术，从文献中检索到 133 例病例。并非所有临床病理数据都可用：76/138 例为男性（55%），中位年龄为 45 岁[四分位距（IQR）34-56]，中位肿瘤大小为 3 厘米（IQR 2-5）。常见发病部位为手（24%）、手指（23%）和足（20%）。14/82 例（17%）患者在诊断时存在疼痛，疼痛持续时间中位数为 7 个月（IQR 3-12）。88 例（74%）患者因疑似良性肿瘤而接受手术。45/71 例（63%）手术不完全切除；45 例中有 32 例（71%）进行了二次切除。在中位随访 26 个月时，118 例患者中有 26 例（22%）出现疾病复发；复发的中位时间为 15 个月（IQR 7-26）。1、3 和 5 年的累积无复发生存率（RFS）分别为 93%、72%和 67%。单因素分析显示，仅症状持续时间在 6 个月或更短与较差的 RFS 显著相关（p=0.046）。4 例（3%）患者发生淋巴结和/或肺部转移。
临床病理表现证实了 MIFS 的低度恶性。然而，局部复发确实存在，患者可能会受到具有潜在远处转移风险的侵袭性形式的影响。强烈建议进行随访。
