Department of Radiation Oncology, University of Florida College of Medicine, Gainesville, Florida.
Department of Radiation Oncology, University of Florida College of Medicine, Jacksonville, Florida.
Int J Radiat Oncol Biol Phys. 2018 Mar 15;100(4):997-1003. doi: 10.1016/j.ijrobp.2017.12.259. Epub 2017 Dec 21.
Radiation therapy (RT) is often used in the treatment of unresectable or recurrent aggressive fibromatosis (also known as desmoid tumor) typically with excellent local control. Prior reports have suggested that local control in pediatric patients with aggressive fibromatosis is poor. We aimed to report a long-term single-institution experience with the radiotherapeutic treatment of these tumors with a focus on age-dependent outcomes.
A total of 101 patients treated with RT for aggressive fibromatosis between 1975 and 2015 at a single institution were identified. A variety of demographic and treatment-related variables were abstracted from patients' medical records. Kaplan-Meier analyses were performed to investigate the relationship between these variables and local control.
Overall survival was excellent (98% and 95% at 5 and 10 years, respectively); local control was likewise excellent (82% and 78% at 5 and 10 years, respectively). Patients aged <20 years at diagnosis had significantly worse 5-year local control than those aged >40 years at diagnosis (72% vs 97%; hazard ratio, 9.0; P = .009). Patients treated with once-daily fractionation had significantly improved 5-year local control compared with those treated with twice-daily fractionation (90% vs 73%; hazard ratio, 0.3; P = .008). Neither the presence of gross versus microscopic residual disease, initial versus recurrent presentation, number of prior surgical procedures, nor tumor size had any effect on 5-year local control. In a total of 36.6% of patients, Common Terminology Criteria for Adverse Events grade 3 or 4 toxicity developed following treatment; the frequency of toxicities was reduced in patients treated during or after 1995 (24.5%) relative to those treated prior to 1995 (51.9%, P = .02).
RT for aggressive fibromatosis offers excellent local control and should remain the standard of care for patients with unresectable or recurrent disease. Younger patients have diminished local control relative to older patients, suggesting possible biological differences contributing to radioresistance in the pediatric and young adult population.
放射治疗（RT）常用于治疗无法切除或复发性侵袭性纤维瘤病（也称为硬纤维瘤），通常可实现极佳的局部控制。先前的报告表明，儿童侵袭性纤维瘤病患者的局部控制效果较差。我们旨在报告单机构长期使用放射疗法治疗这些肿瘤的经验，并重点关注年龄相关的结果。
在一家机构中，从 1975 年至 2015 年，共确定了 101 例接受 RT 治疗侵袭性纤维瘤病的患者。从患者的病历中提取了各种人口统计学和治疗相关变量。通过 Kaplan-Meier 分析研究这些变量与局部控制之间的关系。
总生存率非常高（分别为 5 年和 10 年时的 98%和 95%）；局部控制同样很好（分别为 5 年和 10 年时的 82%和 78%）。诊断时年龄<20 岁的患者与诊断时年龄>40 岁的患者相比，5 年局部控制率明显较差（72%对 97%；危险比，9.0；P=.009）。每天一次分割治疗的患者与每天两次分割治疗的患者相比，5 年局部控制率显著提高（90%对 73%；危险比，0.3；P=.008）。无论是否存在大体与镜下残留疾病、初始与复发表现、手术次数或肿瘤大小如何，均不会影响 5 年局部控制率。在总共 36.6%的患者中，治疗后出现常见不良事件术语标准 3 级或 4 级毒性；与 1995 年之前接受治疗的患者（51.9%）相比，1995 年或之后接受治疗的患者（24.5%）的毒性频率降低（P=.02）。
RT 治疗侵袭性纤维瘤病可实现极佳的局部控制，应继续作为不可切除或复发性疾病患者的标准治疗方法。与年龄较大的患者相比，年轻患者的局部控制率降低，这表明儿科和年轻成人人群中可能存在导致放射抵抗的生物学差异。
