Abstract：
OBJECTIVE: This article discusses the evolving approaches to desmoid tumors management, shedding light on recent developments.
RESULTS: Active surveillance has become the primary approach for managing primary peripheral desmoid tumors. This strategy was initially based on evidence from retrospective studies. Roughly 50% of cases managed with active surveillance show spontaneous stabilization or regression. Recent prospective trials conducted in Italy, The Netherlands, and France (2022-2023) confirm the efficacy of active surveillance, revealing 3-year progression-free survival rates ranging from 53.4 to 58%. For the patients under active surveillance, decisions regarding treatment are based on significant tumor growth or progressive symptoms. Moreover, three contemporary randomized trials investigated medical treatments for progressive or recurrent desmoid tumors. Sorafenib, pazopanib, and nirogacestat demonstrated clinical activity, as evidenced by favorable progression-free survival and objective response rates.
CONCLUSIONS: Active surveillance has solidified its position as the primary management approach for desmoid tumors, validated by three robust prospective studies. Three recent randomized trials explored medical treatment for progressive or recurrent desmoid tumors, revealing promising clinical activities.
RESULTS: Active surveillance has become the primary approach for managing primary peripheral desmoid tumors. This strategy was initially based on evidence from retrospective studies. Roughly 50% of cases managed with active surveillance show spontaneous stabilization or regression. Recent prospective trials conducted in Italy, The Netherlands, and France (2022-2023) confirm the efficacy of active surveillance, revealing 3-year progression-free survival rates ranging from 53.4 to 58%. For the patients under active surveillance, decisions regarding treatment are based on significant tumor growth or progressive symptoms. Moreover, three contemporary randomized trials investigated medical treatments for progressive or recurrent desmoid tumors. Sorafenib, pazopanib, and nirogacestat demonstrated clinical activity, as evidenced by favorable progression-free survival and objective response rates.
CONCLUSIONS: Active surveillance has solidified its position as the primary management approach for desmoid tumors, validated by three robust prospective studies. Three recent randomized trials explored medical treatment for progressive or recurrent desmoid tumors, revealing promising clinical activities.
摘要：
目的：本文讨论了不断发展的硬纤维瘤治疗方法，揭示了最近的事态发展。
结果：主动监测已成为治疗原发性外周硬纤维瘤的主要方法。该策略最初基于回顾性研究的证据。通过主动监测管理的病例中约有50％表现出自发稳定或消退。最近在意大利进行的前瞻性试验，荷兰,法国（2022-2023年）确认了主动监测的有效性，显示3年无进展生存率为53.4%至58%.对于积极监测的患者，关于治疗的决定是基于显著的肿瘤生长或进行性症状。此外，3项当代随机试验研究了药物治疗进展性或复发性硬纤维瘤.索拉非尼,帕唑帕尼,nirogacestat表现出临床活性，有利的无进展生存期和客观缓解率证明了这一点。
结论：主动监测巩固了其作为硬纤维瘤主要治疗方法的地位，通过三项稳健的前瞻性研究进行了验证。最近三项随机试验探索了药物治疗进展性或复发性硬纤维瘤，揭示了有希望的临床活动。
结果：主动监测已成为治疗原发性外周硬纤维瘤的主要方法。该策略最初基于回顾性研究的证据。通过主动监测管理的病例中约有50％表现出自发稳定或消退。最近在意大利进行的前瞻性试验，荷兰,法国（2022-2023年）确认了主动监测的有效性，显示3年无进展生存率为53.4%至58%.对于积极监测的患者，关于治疗的决定是基于显著的肿瘤生长或进行性症状。此外，3项当代随机试验研究了药物治疗进展性或复发性硬纤维瘤.索拉非尼,帕唑帕尼,nirogacestat表现出临床活性，有利的无进展生存期和客观缓解率证明了这一点。
结论：主动监测巩固了其作为硬纤维瘤主要治疗方法的地位，通过三项稳健的前瞻性研究进行了验证。最近三项随机试验探索了药物治疗进展性或复发性硬纤维瘤，揭示了有希望的临床活动。
