The Princess Alexandra Hospital Department of Medical Imaging, Brisbane, Queensland, Australia.
J Med Imaging Radiat Oncol. 2022 Sep;66(6):812-818. doi: 10.1111/1754-9485.13456. Epub 2022 Jul 16.
Ewing sarcoma (ES) is a small round cell sarcoma which usually occurs in the paediatric and young adult age groups. This pictorial review briefly discusses and presents the typical skeletal and extraskeletal locations which ES is encountered, including the metadiaphysis of long bones, pelvis, chest wall and paravertebral regions. Five cases of ES in atypical locations are then presented along with an observation of demographic differences between skeletal and extraskeletal ES. These cases include ES arising from the metatarsal, scapula, breast, intracranial soft tissue and the paranasal sinuses. Presenting symptoms, treatment approach and prognosis are highly variable depending on the location; and multimodal imaging is centrepiece in the management process. Management usually includes any combination of chemotherapy, radiotherapy and surgery. The WHO classification system was updated in 2020 to include some of the rare genetic subtypes of ES. One such case of the BCOR subtype is demonstrated in this review and occurs in an atypical location. Whether these subtypes have distinct radiological features or a propensity to occur in atypical locations is an area for further research.
尤因肉瘤（ES）是一种小圆细胞肉瘤，通常发生在儿童和青年人群中。本文通过典型的骨骼和骨骼外部位的病例图片简要讨论并介绍了 ES 的发病部位，包括长骨骨干骺端、骨盆、胸壁和椎旁区。然后，我们展示了五个位于非典型部位的 ES 病例，并观察了骨骼外和骨骼 ES 之间的人口统计学差异。这些病例包括发生于跖骨、肩胛骨、乳房、颅内软组织和副鼻窦的 ES。临床表现、治疗方法和预后高度取决于发病部位，多模态成像在管理过程中处于核心地位。治疗方法通常包括化疗、放疗和手术的任何组合。世界卫生组织（WHO）分类系统于 2020 年更新，纳入了 ES 的一些罕见基因亚型。在本综述中展示了其中一种 BCOR 亚型，它发生在一个非典型部位。这些亚型是否具有独特的影像学特征或倾向于发生在非典型部位，这是一个有待进一步研究的领域。
