Arnhem Radiotherapy Institute, Arnhem.
Department of Clinical Oncology, LUMC, Leiden, The Netherlands.
Ann Oncol. 2013 Oct;24(10):2672-2676. doi: 10.1093/annonc/mdt254. Epub 2013 Jul 17.
To determine the activity of radiotherapy in patients with inoperable desmoid-type fibromatosis (DF) a multicenter prospective phase II trial was carried out.
Patients with inoperable progressive disease of primary, recurrent or incompletely resected lesions received a dose of 56 Gy in 28 fractions. Follow-up MRI studies were carried out every 3 months for 2 years and thereafter every 6 months. The primary end point was local control rate at 3 years, estimated by a nonparametric method for interval-censored survival data. Secondary end points were objective tumor response, acute and late toxic effect.
Forty-four patients (27 F/17 M) were enrolled from 2001 to 2008. Median age was 39.5 years. Main tumor sites included trunk 15 (34.1%) and extremities 27 (61.3%). Median follow-up was 4.8 years. The 3-year local control rate was 81.5% (90% one-sided confidence interval 74% to 100%). Best overall response during the first 3 years was complete response (CR) 6 (13.6%), partial response (PR) 16 (36.4%), stable disease 18 (40.9%), progressive disease 3 (6.8%) and nonassessable 1 (2.3%). Five patients developed new lesions. After 3 years, the response further improved in three patients: (CR 2, PR 1). Acute grade 3 side-effects were limited to skin, mucosal membranes and pain. Late toxic effect consisted of mild edema in 10 patients.
Moderate dose radiotherapy is an effective treatment of patients with DF. Response after radiation therapy is slow with continuing regression seen even after 3 years.
为了确定不可手术的硬纤维瘤（DF）患者的放疗活性，进行了一项多中心前瞻性 II 期试验。
患有原发性、复发性或不完全切除病变的不可手术进行性疾病的患者接受 56 Gy 的剂量，分为 28 个部分。在最初的 2 年内每 3 个月进行一次 MRI 随访研究，此后每 6 个月进行一次。主要终点是通过非参数方法估计的 3 年局部控制率，用于间隔censored 生存数据。次要终点是客观肿瘤反应、急性和晚期毒性效应。
2001 年至 2008 年期间，共招募了 44 名患者（27 名女性/17 名男性）。中位年龄为 39.5 岁。主要肿瘤部位包括躯干 15 例（34.1%）和四肢 27 例（61.3%）。中位随访时间为 4.8 年。3 年局部控制率为 81.5%（90%单侧置信区间为 74%至 100%）。在最初的 3 年内，最佳总反应为完全缓解（CR）6 例（13.6%）、部分缓解（PR）16 例（36.4%）、稳定疾病 18 例（40.9%）、进展疾病 3 例（6.8%）和无法评估 1 例（2.3%）。5 例患者出现新病变。3 年后，3 例患者的反应进一步改善：（CR 2 例，PR 1 例）。急性 3 级副作用仅限于皮肤、黏膜和疼痛。晚期毒性反应包括 10 例轻度水肿。
中等剂量放疗是治疗 DF 患者的有效方法。放疗后的反应缓慢，甚至在 3 年后仍可见持续消退。
