Penel Nicolas, Le Cesne Axel, Bonvalot Sylvie, Giraud Antoine, Bompas Emmanuelle, Rios Maria, Salas Sébastien, Isambert Nicolas, Boudou-Rouquette Pascaline, Honore Charles, Italiano Antoine, Ray-Coquard Isabelle, Piperno-Neumann Sophie, Gouin François, Bertucci François, Ryckewaert Thomas, Kurtz Jean-Emmanuel, Ducimetiere Françoise, Coindre Jean-Michel, Blay Jean-Yves
Medical Oncology Department, Centre Oscar Lambret, Lille, France.
Department of Medical Oncology & Surgery, Institut Gustave Roussy, Villejuif, France.
Eur J Cancer. 2017 Sep;83:125-131. doi: 10.1016/j.ejca.2017.06.017. Epub 2017 Jul 20.
The outcome of desmoid-type fibromatosis (DTF) is unpredictable. Currently, a wait-and-see approach tends to replace large en bloc resection as the first therapeutic approach. Nevertheless, there are no validated factors to guide the treatment choice.
We conducted a prospective study of 771 confirmed cases of DTF. We analysed event-free survival (EFS) based on the occurrence of relapse after surgery, progressive disease during the wait-and-see approach, or change in therapeutic strategy. Identification of prognostic factors was performed using classical methods (log-rank test and Cox model).
Overall, the 2-year EFS was 56%; this value did not differ between patients undergoing an operation and those managed by the wait-and-see approach (53% versus 58%, p = 0.415). In univariate analysis, two prognostic factors significantly influenced the outcome: the nature of diagnostic sampling (p = 0.466) and primary location (p = 0.0001). The 2-year EFS was only 32% after open biopsy. The 2-year EFS was 66% for favourable locations (abdominal wall, intra-abdominal, breast, digestive viscera and lower limb) and 41% for unfavourable locations. Among patients with favourable locations, the 2-year EFS was similar in patients treated by both surgery (70%) and the wait-and-see approach (63%; p = 0.413). Among patients with unfavourable locations, the 2-year EFS was significantly enhanced in patients initially managed with the wait-and-see approach (52%) compared with those who underwent initial surgery (25%; p = 0.001).
The location of DTF is a major prognostic factor for EFS. If these findings are confirmed by independent analysis, personalised management of DTF must consider this easily obtained parameter.
韧带样型纤维瘤病（DTF）的预后不可预测。目前，观察等待策略倾向于取代大块整块切除成为首选治疗方法。然而，尚无经过验证的因素可指导治疗选择。
我们对771例确诊的DTF病例进行了一项前瞻性研究。我们根据手术后复发、观察等待期间疾病进展或治疗策略的改变来分析无事件生存期（EFS）。使用经典方法（对数秩检验和Cox模型）进行预后因素的识别。
总体而言，2年EFS为56%；接受手术的患者与采用观察等待策略的患者之间这一数值无差异（53%对58%，p = 0.415）。单因素分析中，两个预后因素对结果有显著影响：诊断性取材的性质（p = 0.466）和原发部位（p = 0.0001）。开放活检后的2年EFS仅为32%。有利部位（腹壁、腹腔内、乳腺、消化脏器和下肢）的2年EFS为66%，不利部位为41%。在有利部位的患者中，接受手术治疗（70%）和观察等待策略治疗（63%；p = 0.413）的患者2年EFS相似。在不利部位的患者中，与最初接受手术的患者（25%）相比，最初采用观察等待策略的患者2年EFS显著提高（52%；p = 0.001）。
DTF的部位是EFS的主要预后因素。如果这些发现得到独立分析的证实，DTF的个体化管理必须考虑这个易于获得的参数。
