Xiong Bo, Chen Min, Ye Feng, Zhang Zhuxue, Yin Lijuan, Huang Huifen, Chen Huijiao, Zhang Hongying
1 Department of Pathology, West China Hospital, Sichuan University, 610041 Chengdu, Sichuan, China.
Pediatr Dev Pathol. 2013 Sep-Oct;16(5):353-6. doi: 10.2350/13-04-1327-CR.1. Epub 2013 Jul 11.
Synovial sarcoma originating in the liver is extremely rare, and thus far only 3 cases have been reported in the English literature. Herein, we report a primary hepatic synovial sarcoma in a 13-year-old Chinese boy. This patient present with a 10-day right upper quadrant pain, and a heterogeneous mass was documented in the right hepatic lobe by computed tomography. Subsequently, the patient underwent right hepatectomy. Histologically, the tumor exhibited classic features of monophasic synovial sarcoma. The diagnosis was confirmed by the presence of SS18 gene rearrangement and identification of SS18-SSX1 fusion transcript. Unfortunately, a relapsing mass was detected 11 months after the surgery. To the best of our knowledge, the current case is the 1st published example in the pediatric population.
起源于肝脏的滑膜肉瘤极为罕见，迄今为止英文文献中仅报道过3例。在此，我们报告1例13岁中国男孩的原发性肝脏滑膜肉瘤。该患者因右上腹疼痛10天就诊，计算机断层扫描显示右肝叶有一不均匀肿块。随后，患者接受了右肝切除术。组织学上，肿瘤表现出单相滑膜肉瘤的典型特征。通过检测到SS18基因重排以及鉴定出SS18-SSX1融合转录本确诊。不幸的是，术后11个月发现复发肿块。据我们所知，目前该病例是儿科人群中首个发表的病例。
