过去，人们认为BCOR-CCNB3肉瘤与尤文肉瘤类似; 然而，当前的研究已经证明它实际上是一种与之不同的疾病类型，现在在未分化圆细胞肉瘤的不同实体下进行分类。这种肿瘤最常见于青少年和年轻成年人，男性更为普遍。它可以形成在骨骼和软组织中，并且在骨盆，下肢和脊柱旁区域中最常见。在这里，我们描述一个12岁男孩右大腿近端后部的软组织肿瘤的案例，后来通过遗传学分析证明是BCOR-CCNB3融合。版权所有©2023，Kedia等。

In the past, BCOR-CCNB3 sarcoma was believed to be comparable to Ewing's sarcoma; however, current research has proven that it is, in reality, a distinct type of the illness, now classified as a distinct entity under undifferentiated round cell sarcomas. This tumour most frequently affects teenagers and young adults, and it is more prevalent in men. It can form in both bone and soft tissue, and it appears most frequently in the pelvis, lower extremities, and paraspinal region. Here, we describe a case of a soft tissue tumour in the proximal posterior portion of the right thigh of a 12-year-old male that was subsequently shown to be a BCOR-CCNB3 fusion using genetic analysis.Copyright © 2023, Kedia et al.