Department of Surgery, Sarcoma Unit, Royal Marsden Hospital, London, UK.
Department of Surgery, Netherlands Cancer Institute, Amsterdam, The Netherlands.
Ann Surg Oncol. 2019 Dec;26(13):4699-4706. doi: 10.1245/s10434-019-07826-6. Epub 2019 Sep 17.
The behavior of desmoid tumors is unpredictable and varies from spontaneous remission to symptomatic and radiologic progression. This study aimed to evaluate the radiologic and symptomatic course of the disease in patients initially managed with active surveillance.
Patients with a primary desmoid tumor at any anatomic location diagnosed between 1998 and 2016 were identified in a prospectively maintained database from a single sarcoma reference center in the United Kingdom. Inverse univariate Cox proportional hazard regression analyses were conducted to evaluate the course of the disease and indications for initiating treatment.
The study identified 168 patients with a primary desmoid tumor initially managed with active surveillance. The tumors were located in the abdominal wall (n = 61, 36%), an extremity (n = 51, 30%), chest wall (n = 30, 18%), intra-abdominal site (n = 15, 9%), or elsewhere (n = 11, 6%). Of all the patients, 36% experienced radiologic progressive disease, 36% had stable disease, and 27% regressed. The patients younger than 50 years were more likely to progress (p = 0.046), whereas the patients with chest wall or upper-extremity tumors reported significantly more pain (p = 0.01). Eventually, 46% of the patients proceeded to treatment. The median time to start of treatment after initial surveillance was 31 months, whereas the median follow-up time for the patients not receiving any treatment was 40.5 months. The indications for initiation of treatment were pain (32%), progression (31%), or both (13%).
Patients with desmoid tumors can be managed with initial active surveillance, although almost half of patients may eventually need treatment. Pain, tumor progression, or both are the most common indications for the initiation of treatment.
硬纤维瘤的行为不可预测，从自发缓解到有症状和影像学进展不等。本研究旨在评估最初接受主动监测治疗的患者的疾病影像学和症状进程。
从英国一家肉瘤参考中心前瞻性维护的数据库中确定了 1998 年至 2016 年间诊断为任何解剖部位原发性硬纤维瘤的患者。进行逆单变量 Cox 比例风险回归分析，以评估疾病进程和开始治疗的指征。
本研究确定了 168 例最初接受主动监测治疗的原发性硬纤维瘤患者。肿瘤位于腹壁（n=61，36%）、四肢（n=51，30%）、胸壁（n=30，18%）、腹腔内部位（n=15，9%）或其他部位（n=11，6%）。所有患者中，36%的患者影像学疾病进展，36%的患者疾病稳定，27%的患者疾病消退。50 岁以下的患者更有可能进展（p=0.046），而胸壁或上肢肿瘤患者报告的疼痛明显更多（p=0.01）。最终，46%的患者接受了治疗。最初监测后开始治疗的中位时间为 31 个月，而未接受任何治疗的患者的中位随访时间为 40.5 个月。开始治疗的指征是疼痛（32%）、进展（31%）或两者兼有（13%）。
可以对硬纤维瘤患者进行初始主动监测治疗，尽管近一半的患者最终可能需要治疗。疼痛、肿瘤进展或两者兼而有之是开始治疗的最常见指征。
