Department of Radiology, Great Ormond Street Hospital for Children, London, UK.
Department of Radiology, Great Ormond Street Hospital for Children, London, UK.
Clin Radiol. 2022 Jul;77(7):e532-e539. doi: 10.1016/j.crad.2022.03.023. Epub 2022 Apr 30.
To highlight the imaging findings in a case series of histologically confirmed infantile fibrosarcoma (IF) and identify any features specific to this entity.
Retrospective identification was undertaken of patients with histologically confirmed IF from the electronic patient databases of two institutions between 1 January 2010 and 1 May 2021. Available pre-treatment imaging, histopathological reports, and clinical records were reviewed.
Eighteen patients with IF met the inclusion criteria. There were 10 male and eight female patients with a mean age at presentation of 3 weeks. All patients had the t (12; 15) chromosomal translocation. Eleven (61%) tumours were located in the extremities, three were in the craniofacial region, two were intrathoracic, one abdominal and one paraspinal. A single patient had extensive metastases. The tumours were generally isointense to skeletal muscle on T1-weighted sequences and hyperintense on T2 with heterogeneous enhancement and high cellularity seen as diffusion restriction. Fifteen of the 18 lesions were evaluated on ultrasound and appeared as heterogeneous, hypervascular solid or mixed solid/cystic masses, mimicking benign vascular lesions in two cases.
The present two-centre, retrospective study of the largest case series described thus far demonstrates that IF is always highly cellular on magnetic resonance imaging but has no other specific imaging features. It should be considered in the differential diagnosis of any enlarging soft-tissue, solid mass arising in the limbs or neck at birth or in infancy.
突出组织学证实的婴儿纤维肉瘤(IF)病例系列的影像学表现,并确定该实体特有的任何特征。
回顾性确定了 2010 年 1 月 1 日至 2021 年 5 月 1 日期间两家机构电子患者数据库中经组织学证实的 IF 患者。回顾了可用的治疗前影像学、组织病理学报告和临床记录。
18 名 IF 患者符合纳入标准。10 名男性和 8 名女性,平均发病年龄为 3 周。所有患者均存在 t(12;15)染色体易位。11 个(61%)肿瘤位于四肢,3 个位于头面部,2 个位于胸部,1 个位于腹部,1 个位于脊柱旁。单一患者有广泛转移。肿瘤在 T1 加权序列上与骨骼肌等信号强度,在 T2 上呈高信号,增强不均匀,高细胞性表现为弥散受限。18 个病变中有 15 个进行了超声评估,表现为不均匀、高血管性实性或混合性实性/囊性肿块,在 2 例中类似于良性血管病变。
目前对迄今为止描述的最大病例系列进行的两中心回顾性研究表明,IF 在磁共振成像上始终具有高度细胞性,但没有其他特定的影像学特征。在出生时或婴儿期四肢或颈部任何增大的软组织、实性肿块的鉴别诊断中应考虑 IF。