Park K, van Rijn R, McHugh K
Radiology Department, Great Ormond Street Hospital for Children, London, WC1N 3JH, UK.
Cancer Imaging. 2008 Apr 22;8(1):102-15. doi: 10.1102/1470-7330.2008.0014.
Paediatric soft tissue sarcomas (STS) are a group of malignant tumours that originate from primitive mesenchymal tissue and account for 7% of all childhood tumours. Rhabdomyosarcomas (RMS) and undifferentiated sarcomas account for approximately 50% of soft tissue sarcomas in children and non-rhabdomyomatous soft tissue sarcomas (NRSTS) the remainder. The prognosis and biology of STS tumours vary greatly depending on the age of the patient, the primary site, tumour size, tumour invasiveness, histologic grade, depth of invasion, and extent of disease at diagnosis. Over recent years, there has been a marked improvement in survival rates in children and adolescents with soft tissue sarcoma and ongoing international studies continue to aim to improve these survival rates whilst attempting to reduce the morbidity associated with treatment. Radiology plays a crucial role in the initial diagnosis and staging of STS, in the long term follow-up and in the assessment of many treatment related complications. We review the epidemiology, histology, clinical presentation, staging and prognosis of soft tissue sarcomas and discuss the role of radiology in their management.
小儿软组织肉瘤（STS）是一组起源于原始间叶组织的恶性肿瘤，占所有儿童肿瘤的7%。横纹肌肉瘤（RMS）和未分化肉瘤约占儿童软组织肉瘤的50%，其余为非横纹肌瘤性软组织肉瘤（NRSTS）。STS肿瘤的预后和生物学特性因患者年龄、原发部位、肿瘤大小、肿瘤侵袭性、组织学分级、浸润深度以及诊断时疾病范围的不同而有很大差异。近年来，儿童和青少年软组织肉瘤的生存率有了显著提高，正在进行的国际研究继续致力于提高这些生存率，同时试图降低与治疗相关的发病率。放射学在STS的初始诊断和分期、长期随访以及许多治疗相关并发症的评估中起着至关重要的作用。我们综述了软组织肉瘤的流行病学、组织学、临床表现、分期和预后，并讨论放射学在其管理中的作用。
