Mathew Denny, Prince Daniel N, Mahomed Nasreen
Diagnostic Radiology, University of the Witwatersrand, Johannesburg, South Africa.
Department of Radiology, Rahima Moosa Mother and Child Hospital, University of the Witwatersrand, Johannesburg, South Africa.
SA J Radiol. 2019 Jun 27;23(1):1733. doi: 10.4102/sajr.v23i1.1733. eCollection 2019.
Chest wall or pleural-based tumours represent a heterogeneous group of lesions that are infrequent in children and infants; however, a large proportion of these lesions are malignant in nature. Categorising them on the basis of primary versus secondary, site of origin (osseous and cartilage, or soft tissue) and tissue composition may assist in narrowing the differential diagnosis. We present a case of a 7-year-old boy with a progressive history of dyspnoea. The initial chest radiograph (CXR) demonstrated complete opacification of the left hemithorax with no air bronchograms. This was associated with the cut-off of the left main bronchus and mediastinal shift to the right. The post-contrast computed tomography (CT) of the chest showed multiple left-sided enhancing pleural-based masses with collapse of the left lung. These lesions were locally invasive as demonstrated by the intra and extra-thoracic extension. There were no associated erosions of the adjacent ribs or intra-tumoural calcifications. Based on the imaging findings, the diagnosis of extra-skeletal Ewing sarcoma (ES-EWS) of the chest wall was made with a differential diagnosis of rhabdomyosarcoma. A core biopsy was performed of the pleural-based mass, and histology with immunohistochemistry confirmed the diagnosis of a malignant small round blue cell tumour; subtype Ewing sarcoma family tumour (ESFT). The child was subsequently commenced on chemotherapy. The diagnosis of ES-EWS should be considered when a child or adolescent presents with an ill-defined, eccentric, chest wall mass in the absence of a lesion with a primary osseous origin. Imaging plays a key role in tumour staging, therapeutic planning and follow-up of patients.
胸壁或胸膜源性肿瘤是一组异质性病变，在儿童和婴儿中并不常见；然而，这些病变中很大一部分本质上是恶性的。根据原发性与继发性、起源部位（骨和软骨或软组织）以及组织构成进行分类，可能有助于缩小鉴别诊断范围。我们报告一例7岁男孩，有进行性呼吸困难病史。最初的胸部X线片（CXR）显示左半胸完全致密，无空气支气管征。这与左主支气管截断及纵隔向右移位有关。胸部增强计算机断层扫描（CT）显示左侧多个强化的胸膜源性肿块，左肺萎陷。这些病变呈局部浸润性，表现为胸内和胸外扩展。相邻肋骨无相关侵蚀，肿瘤内无钙化。根据影像学表现，诊断为胸壁骨外尤文肉瘤（ES-EWS），鉴别诊断为横纹肌肉瘤。对胸膜源性肿块进行了核心活检，组织学及免疫组化证实为恶性小圆蓝细胞肿瘤；亚型为尤文肉瘤家族肿瘤（ESFT）。该患儿随后开始化疗。当儿童或青少年出现界限不清、偏心的胸壁肿块且无原发性骨源性病变时，应考虑ES-EWS的诊断。影像学在肿瘤分期、治疗计划制定及患者随访中起关键作用。
