背景:乳腺纤维瘤病是一种以单克隆成纤维细胞增殖为特征的罕见疾病。它没有转移能力,但局部复发率高,经常浸润周围组织。手术治疗是参考,但是最近,新的靶向疗法已经出现。我们报告了一名接受独家治疗的乳腺纤维瘤病患者的原始病例。我们的目的是根据临床和放射学结果分析这些治疗方法,医源性影响,和药理作用。
方法:我们报告了一个19岁女性的病例,该患者发展为右乳房下内象限的纤维瘤,测量为50×25毫米(即,体积为27.4cm3)。由于潜在的审美和功能偏见,最初的手术是不可能的。因此,她接受了独家治疗,包括几行:NSAIDs与他莫昔芬和曲普瑞林,其次是索拉非尼,然后是干扰素α2b,最后是舒尼替尼。酪氨酸激酶抑制剂(TKIs)(舒尼替尼),观察到显著的部分反应(最大肿瘤体积减少57%).对于每一种治疗,我们提供了与已知药理作用相关的临床和放射学结果.
结论:TKI是初始手术的一个有趣的替代选择,提供至少部分响应,并可能允许较少的残缺手术。然而,没有药理机制可以明确解释TKI的疗效。总的来说,乳腺纤维瘤病应与肿瘤学家和介入放射科医师一起以跨学科的方式进行治疗,从而为这种特殊的纤维瘤病定位提供了适应性治疗。
BACKGROUND: Breast fibromatosis is a rare disease characterized by monoclonal fibroblast proliferation. It has no ability to metastasize but has a high local recurrence rate and often infiltrates surrounding tissues. Surgical treatment is the reference, but recently, new targeted therapies have emerged. We report an original case of a patient with breast fibromatosis who received exclusive medical treatment. Our aim was to analyze these treatments based on the clinical and radiological outcome, iatrogenic effects, and pharmacological action.
METHODS: We report the case of a 19-year-old woman who developed a desmoid-type fibromatosis of the lower inner quadrant of the right breast, measuring 50 × 25 mm (i.e., a volume of 27.4 cm3). Initial surgery was not possible because of potential esthetic and functional prejudice. Thus, she had an exclusive medical treatment including several lines: NSAIDs with tamoxifen and triptorelin, followed by sorafenib, then interferon α2b, and finally sunitinib. With tyrosine-kinase inhibitors (TKIs) (sunitinib), a significant partial response was observed (57% reduction of the maximal tumoral volume). For each treatment, we provided the clinical and radiological outcome in association with known pharmacological action.
CONCLUSIONS: TKI had been an interesting alternative option to initial surgery, providing at least a partial response and potentially allowing less mutilating surgery. However, no pharmacological mechanism can unequivocally explain TKI efficacy. In general, breast fibromatosis should be treated along with oncologist and interventional radiologists in a trans-disciplinary modality, thus offering an adapted treatment for this particular desmoid-type fibromatosis localization.