Department of Pathology, West China Hospital, Sichuan University, Chengdu 610041, Sichuan, China.
Cardiovasc Pathol. 2013 Nov-Dec;22(6):501-2. doi: 10.1016/j.carpath.2013.04.004. Epub 2013 May 20.
Synovial sarcoma originating in the pulmonary valve is extremely rare. Herein, we report a poorly differentiated synovial sarcoma arising from this peculiar location in a 17-year-old Chinese boy. Histologically, this tumor was entirely poorly differentiated with uniform small round cell morphology, and it exhibited prominent myxoid change in some areas. The diagnosis was confirmed by the presence of SS18 rearrangement and identification of the SS18-SSX1 fusion transcript. To the best of our knowledge, the present case is the first published example of synovial sarcoma occurring in the pulmonary valve. Additionally, this is the first case showing entirely uniform small round cell morphology without classic areas of synovial sarcoma.
发生于肺动脉瓣的滑膜肉瘤极其罕见。在此，我们报道了一例发生于 17 岁中国男孩这一特殊部位的低分化滑膜肉瘤。组织学上，该肿瘤完全为低分化，形态均一呈小圆细胞状，部分区域具有明显黏液样变。通过存在 SS18 重排和识别 SS18-SSX1 融合转录本，明确了诊断。据我们所知，本例是首例报道的发生于肺动脉瓣的滑膜肉瘤。此外，这也是首例完全表现为均一的小圆细胞形态而无经典滑膜肉瘤区域的病例。
