Xu Rui-Fang, He En-Hui, Yi Zhan-Xiong, Lin Jun, Zhang Yan-Ning, Qian Lin-Xue
Department of Ultrasound, Beijing Friendship Hospital, Capital Medical University, Beijing 100050, China.
Department of Urological Surgery, Beijing Friendship Hospital, Capital Medical University, Beijing 100050, China.
World J Clin Cases. 2019 Jul 6;7(13):1677-1685. doi: 10.12998/wjcc.v7.i13.1677.
Primary renal synovial sarcoma (PRSS) is an extremely rare tumor with a poor prognosis. Its imaging and immunohistochemical characteristics may overlap with other renal tumors, which renders its early diagnosis in a dilemma. The diagnosis of primary renal synovial sarcoma requires histopathology and the confirmation of SYT-SSX gene fusion using molecular techniques. Cases of primary renal synovial sarcoma have been previously reported in the literature. However, to our knowledge, primary renal allograft synovial sarcoma was never described.
A 43-year-old male patient who underwent kidney transplantation 9 months ago came to our hospital for regular follow-up. Traditional ultrasonography revealed multiple hypo-echo neoplasms in the renal allograft. Contrast-enhanced computed tomography (CECT) showed slightly hyper-density masses with slow homogeneous enhancement. Ultrasound-guided biopsy was conducted for accurate pathological diagnosis. The neoplasms were diagnosed as synovial sarcoma by pathological, immunohistochemical, and genetic analyses. Positron emission tomography/CT showed no evidence of metastasis. At approximately one week post biopsy, contrast-enhanced ultrasound was conducted to eliminate active hemorrhage. One month later, CECT showed that the biggest neoplasm grew from 3.3 cm to 5.7 cm in diameter. Parametric imaging was conducted with SonoLiver CAP to conduct further quantitative analysis, which showed that the enhancement pattern was heterogeneous hyper-vascular enhancement. Radical surgical resection of the whole renal allograft and ureter was conducted without additional adjuvant chemotherapy or external radiotherapy. Anlotinib was chosen for targeted therapy with a good response.
We propose multimodality imaging for accurate diagnosis of renal allograft synovial sarcoma especially when it is formed by spindle-shaped cells.
原发性肾滑膜肉瘤（PRSS）是一种极为罕见的肿瘤，预后较差。其影像学和免疫组化特征可能与其他肾肿瘤重叠，这使得其早期诊断陷入困境。原发性肾滑膜肉瘤的诊断需要组织病理学检查以及使用分子技术确认SYT-SSX基因融合。此前文献中已有原发性肾滑膜肉瘤的病例报道。然而，据我们所知，原发性肾移植滑膜肉瘤从未被描述过。
一名9个月前接受肾移植的43岁男性患者来我院进行定期随访。传统超声检查显示移植肾内有多个低回声肿物。增强计算机断层扫描（CECT）显示肿物密度略高，呈均匀缓慢强化。为明确病理诊断进行了超声引导下活检。通过病理、免疫组化和基因分析，这些肿物被诊断为滑膜肉瘤。正电子发射断层扫描/计算机断层扫描（PET/CT）显示无转移证据。活检后约一周，进行了超声造影以排除活动性出血。1个月后，CECT显示最大的肿物直径从3.3 cm增大至5.7 cm。使用SonoLiver CAP进行参数成像以进一步进行定量分析，结果显示强化模式为不均匀高血供强化。对整个移植肾和输尿管进行了根治性手术切除，未进行额外的辅助化疗或外照射放疗。选择安罗替尼进行靶向治疗，效果良好。
我们建议采用多模态成像技术准确诊断肾移植滑膜肉瘤，尤其是当它由梭形细胞构成时。
