IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy,
University of Bologna, Bologna, Italy,
Oncol Res Treat. 2020;43(5):189-195. doi: 10.1159/000506844. Epub 2020 Apr 8.
The aim of this retrospective analysis is to understand the natural history of myxofibrosarcoma (MFS), in particular whether the prognosis can be influenced by histologic grade.
We reviewed 229 adult patients with primary MFS of the limbs. We analyzed disease-specific survival (overall survival [OS]) and local recurrence (LR).
Median age was 70 years (range, 19-92). Sixteen (7.0%) were grade 1, 38 (16.6%) grade 2, and 175 (76.4%) grade 3. A worse OS was found in grade 3 MFS (73.1%) than in grade 2 and 1 MFS (91.9 and 100%, respectively) at 5 years (p = 0.031). Locally recurred MFS had a worse OS (p = 0.018). A better LR-free rate (100% at 5 years) was observed in grade 1 MFS; however, a similar rate was observed between grade 2 and 3 tumors (77.1 and 80.0% at 5 years, respectively, p = 0.412).
Grade 3 MFS has the worst prognosis. Grade 1 MFS have the lowest risk of LR. These data could help identify a high-risk patient group, thus selecting a more careful follow-up for higher-risk patients. Since MFS mostly affects the elderly population, it might be useful to reserve adjuvant treatments (radiotherapy and chemotherapy) to higher-risk patients.
本回顾性分析旨在了解黏液纤维肉瘤（MFS）的自然病程，特别是组织学分级是否会影响预后。
我们回顾了 229 例肢体原发性 MFS 成人患者。我们分析了疾病特异性生存（总生存[OS]）和局部复发（LR）。
中位年龄为 70 岁（范围 19-92 岁）。16 例（7.0%）为 1 级，38 例（16.6%）为 2 级，175 例（76.4%）为 3 级。3 级 MFS 的 OS 明显差于 2 级和 1 级（分别为 73.1%和 91.9%和 100%，p=0.031）。局部复发的 MFS 的 OS 较差（p=0.018）。1 级 MFS 的 LR 无复发生存率更好（5 年时为 100%）；然而，2 级和 3 级肿瘤之间的无复发生存率相似（分别为 77.1%和 80.0%，p=0.412）。
3 级 MFS 的预后最差。1 级 MFS 的 LR 风险最低。这些数据可以帮助识别高危患者群体，从而为高危患者选择更仔细的随访。由于 MFS 主要影响老年人群，因此可能有用保留辅助治疗（放疗和化疗）给高危患者。
