原发性肾滑膜肉瘤一例报告并文献复习

Primary synovial sarcoma of the kidney （literature review with 1 case report）

目的 总结原发性肾滑膜肉瘤的临床表现、病理特点和诊治方法.方法 原发性肾滑膜肉瘤患者1例,男,55岁.因右腰背及上腹部疼痛5 h入院.无尿频、尿急、尿痛、血尿、发热等症状.CT检查示右肾中下极恶性占位可能,累及右肾门、肾动静脉及肝脏.行右肾肿瘤根治性切除术.结果 病理检查：肿瘤大小12.5 cm×11.0 cm×9.0 cm.镜下瘤细胞呈梭形、束状排列,细胞异型,可见核分裂相,部分区域见增生的薄壁血管,肿瘤呈浸润性生长,伴发炎症及大量坏死、出血.免疫组化染色示Vimentin、CD99、Bcl-2阳性,CK阴性.病理诊断为原发性肾滑膜肉瘤.患者术后2个月出现局部复发及肝肺转移,予异环磷酰胺、表阿霉素化疗1疗程,患者不能耐受.术后8个月死亡.结论 原发性肾滑膜肉瘤是一种罕见、高度恶性的肿瘤,预后不良,确诊需依赖病理检查、免疫组织化学及RT-PCR检测.根治性切除辅以化学治疗可行.

Objective To discuss the presentations, pathologic features, diagnosis and treatment of primary synovial sarcoma of the kidney. Methods One case of primary synovial sarcoma of the kidney was reported and the relevant literature was reviewed. A 55-year-old man was admitted with complaint of right abdomen and flank pain for 5 h. Computerized tomography revealed a 12.5 cm × 11.0 cm × 9. 0 cm mass located at the middle and lower pole of the right kidney. The patient was taken radical nephrectomy. Results The diagnosis of primary synovial sarcoma of the kidney in the patient was confirmed by postoperative pathology. Under microscope, tumor was typically mitotically active, monomorphic spindle cells growing in intersecting fascicles or in solid sheets with epithelial differentiation. In some areas a haemangiopericytoma-like pattern was found. Immunohistochemical staining showed that the tumor cells were positive for the markers Vimentin, CD99 and Bcl-2, but CK was negative. The patient died of local recurrence and multi-metastasis at 8 months after surgery. Conclusions Primary synovial sarcoma of the kidney is extremely rare with a high grade of malignancy,and its prognosis is poor. The diagnosis depends on pathological features, Immunohistochemical studies and RT-PCR detection. Radical resection combined with chemicaltherapy is considered to be the most reliable treatment so far.