Department of Thoracic Surgery, National Hospital Organization, Hokkaido Cancer Center, Sapporo, Japan.
Department of Surgical Pathology, Sapporo Medical University Hospital, Sapporo, Japan.
Jpn J Clin Oncol. 2021 Apr 1;51(4):654-656. doi: 10.1093/jjco/hyaa240.
A 60-year-old man had a malignant left lower lung tumour with no metastases and underwent video-assisted thoracoscopic left lower lobectomy and lymphadenectomy. Pathological examination led to a diagnosis of capicua transcriptional repressor (CIC)-rearranged sarcoma. He has had 3.5 years of recurrence-free survival. CIC-rearranged sarcoma is a Ewing-like sarcoma that shows pathological findings similar to Ewing sarcoma. Most of CIC-rearranged sarcoma is CIC-double homeobox 4 protein (DUX4) fusion. Pulmonary CIC-rearranged sarcoma is extremely rare and has an unfavourable prognosis. However, complete resection can produce prognosis of long-term survival, and thus, surgery is an important option.
一位 60 岁男性患有无转移的恶性左下肺肿瘤，接受了电视辅助胸腔镜左下肺叶切除术和淋巴结切除术。病理检查提示为帽状转录阻遏物（CIC）重排肉瘤。他无复发生存 3.5 年。CIC 重排肉瘤是一种类似于尤文肉瘤的 Ewing 样肉瘤，具有与尤文肉瘤相似的病理表现。大多数 CIC 重排肉瘤为 CIC-双同源盒 4 蛋白（DUX4）融合。肺部 CIC 重排肉瘤极为罕见，预后不良。然而，完全切除可产生长期生存的预后，因此手术是一种重要的选择。
