Department of Radiology, Brooke Army Medical Center, 3551 Roger Brooke Drive, Fort Sam Houston, San Antonio, TX 78234, USA.
Department of Pathology, Brooke Army Medical Center, 3551 Roger Brooke Drive, Fort Sam Houston, San Antonio, TX 78234, USA.
Clin Imaging. 2021 Jan;69:213-218. doi: 10.1016/j.clinimag.2020.08.030. Epub 2020 Sep 6.
Desmoid-type fibromatosis (DF) is a rare neoplasm characterized by fibroblastic and myofibroblastic proliferation. While characterized as a benign lesion that does not metastasize, desmoid-type fibromatosis exhibits a wide range of behavior from aggressive local tissue invasion and post-surgical recurrence to spontaneous regression. Tumor regression can occur following systemic medical therapy or rarely may occur in the absence of therapy. We present a case of a 50-year-old female with a left thigh vastus medialis intramuscular mass which underwent imaging work-up and subsequent core needle ultrasound-guided biopsy showing results of desmoid-type fibromatosis. Following biopsy, the tumor showed prompt, complete regression with complete MRI resolution 2 months following biopsy. The patient showed no evidence of disease recurrence out to one year on MRI surveillance. This case report will discuss desmoid-type fibromatosis imaging features, treatment strategies, spectrum of disease behavior, and atypical behavior such as the spontaneous tumor regression as seen in this case report. To our knowledge there have been no reported cases of DF spontaneous regression 2 months following a core needle biopsy. Understanding the variable behavior of desmoid-type fibromatosis can assist the radiologist in guiding management of these lesions with the goal of optimizing clinical outcomes and preventing unnecessary aggressive treatments for stable or regressing disease.
韧带样纤维瘤病(DF)是一种罕见的肿瘤,其特征为成纤维细胞和肌成纤维细胞增殖。尽管被认为是一种良性病变,不会转移,但韧带样纤维瘤病的行为表现范围广泛,从侵袭性局部组织侵犯和手术后复发到自发性消退不等。肿瘤消退可发生在全身药物治疗后,也可在没有治疗的情况下罕见发生。我们报告了 1 例 50 岁女性,左大腿股直肌内肌间肿块,进行了影像学检查,随后进行了核心针超声引导活检,结果显示为韧带样纤维瘤病。活检后,肿瘤迅速完全消退,MRI 分辨率在活检后 2 个月完全恢复。在 MRI 监测的 1 年内,患者未出现疾病复发的迹象。本病例报告将讨论韧带样纤维瘤病的影像学特征、治疗策略、疾病行为谱以及本病例报告中所见的这种自发肿瘤消退的非典型行为。据我们所知,在核心针活检后 2 个月内,DF 自发消退的病例尚未有报道。了解韧带样纤维瘤病的可变行为可以帮助放射科医生指导这些病变的管理,以优化临床结果,并防止对稳定或消退的疾病进行不必要的激进治疗。