PDF(Pubmed)
Abstract：
UNASSIGNED: Breast desmoid-type fibromatosis (BDF) is a rare mesenchymal tumor accounting for only 0.2% of solid breast tumors. It is classified as an intermediate tumor because it is locally aggressive but has no metastatic potential. Its diagnosis is often difficult because it shares many clinical and radiologic aspects with breast carcinomas and therefore relies on anatomopathological analysis which may be supplemented by genetic analysis. The treatment of BDF has considerably evolved in the past years. While surgery was the cornerstone of the management prior to the 2000s, recent data have shown the value of active surveillance (AS) from the time of diagnosis. Indeed, after 2 years of AS, the progression-free survival (PFS) of the disease is identical or superior to surgery. Moreover, spontaneous regression has been observed in 30% of patients undergoing AS. In case of disease progression, surgery can be considered on a case-by-case basis, as well as systemic treatments.
UNASSIGNED: We present a case of bilateral BDF affecting a 20-year-old woman for whom the first suggested treatment was bilateral mastectomy with reconstruction. After a second opinion, the decision was revised and AS was initiated. Almost 3 years after the onset of AS, tumors have shown a continuous regression.
UNASSIGNED: This case demonstrates the need for experience in the management of mesenchymal tumors to avoid overtreatment by mutilating surgeries which promote recurrence. Moreover, to our knowledge, very few cases of bilateral BDF have been published to date. It thus seemed relevant for us to report this rare case which supports the interest of AS for DF, as recently advised by the Desmoid Tumor Working Group guidelines.
UNASSIGNED: We present a case of bilateral BDF affecting a 20-year-old woman for whom the first suggested treatment was bilateral mastectomy with reconstruction. After a second opinion, the decision was revised and AS was initiated. Almost 3 years after the onset of AS, tumors have shown a continuous regression.
UNASSIGNED: This case demonstrates the need for experience in the management of mesenchymal tumors to avoid overtreatment by mutilating surgeries which promote recurrence. Moreover, to our knowledge, very few cases of bilateral BDF have been published to date. It thus seemed relevant for us to report this rare case which supports the interest of AS for DF, as recently advised by the Desmoid Tumor Working Group guidelines.
摘要：
未经证实：乳腺纤维瘤病（BDF）是一种罕见的间叶性肿瘤，仅占乳腺实性肿瘤的0.2％。它被归类为中间肿瘤，因为它是局部侵袭性的，但没有转移潜力。它的诊断通常很困难，因为它与乳腺癌共享许多临床和放射学方面，因此依赖于解剖病理学分析，可以通过遗传分析进行补充。BDF的治疗在过去几年中已经有了相当大的发展。虽然手术是2000年代之前管理的基石，最近的数据显示了从诊断时开始主动监测(AS)的价值.的确,经过两年的AS，该疾病的无进展生存期(PFS)与手术相同或优于手术.此外，在30%的AS患者中观察到自发消退.在疾病进展的情况下，手术可以根据具体情况考虑，以及系统治疗。
UNASSIGNED：我们介绍了一例双侧BDF的病例，该病例影响了一名20岁的女性，其第一个建议的治疗方法是双侧乳房切除术和重建术。在第二意见之后，该决定进行了修订，并启动了AS。AS发病后将近3年，肿瘤已显示出持续消退。
UNASSIGNED：该病例表明需要在间充质肿瘤的治疗方面有经验，以避免因残害手术而导致的过度治疗，从而促进复发。此外，根据我们的知识，迄今为止，很少有双边BDF病例发表。因此，我们似乎有必要报告这个罕见的病例，这支持了AS对DF的兴趣，正如最近由Desmoid肿瘤工作组指南所建议的那样。
UNASSIGNED：我们介绍了一例双侧BDF的病例，该病例影响了一名20岁的女性，其第一个建议的治疗方法是双侧乳房切除术和重建术。在第二意见之后，该决定进行了修订，并启动了AS。AS发病后将近3年，肿瘤已显示出持续消退。
UNASSIGNED：该病例表明需要在间充质肿瘤的治疗方面有经验，以避免因残害手术而导致的过度治疗，从而促进复发。此外，根据我们的知识，迄今为止，很少有双边BDF病例发表。因此，我们似乎有必要报告这个罕见的病例，这支持了AS对DF的兴趣，正如最近由Desmoid肿瘤工作组指南所建议的那样。
