BCOR-CCNB3融合肉瘤是最近描述的未分化肉瘤,具有2个附近基因BCOR和CCNB3的新的复发性倒位。它通常会影响骨盆的骨骼和软组织,四肢,和椎旁区域,并追求可变的临床过程。原发性肾BCOR-CCNB3融合肉瘤非常罕见,只有少数病例被记录在案。准确的诊断通常具有挑战性,由于该实体的稀有性,因此没有任何协议来处理该实体。我们报告了一个16岁男孩的原发性肾BCOR-CCNB3融合肉瘤的发现,并简要回顾了文献。
BCOR-CCNB3 fusion sarcoma is a recently described undifferentiated sarcoma with a novel recurrent inversion of 2 nearby genes BCOR and CCNB3. It typically affects bone and soft tissues of the pelvis, extremity, and paraspinal region and pursues variable clinical course. Primary renal BCOR-CCNB3 fusion sarcoma is very rare, and only a small number of cases have been documented. Accurate diagnosis is often challenging, and there is not any agreement for the treatment of this entity due to its rarity. We report findings of primary renal BCOR-CCNB3 fusion sarcoma in a 16-year-old boy with a brief review of the literature.