肾原发性滑膜肉瘤1例报告及文献复习

Primary renal synovial sarcoma:a metastasized case report and literature review

目的提高对肾原发性滑膜肉瘤的诊治水平。方法回顾性分析1例晚期肾原发性滑膜肉瘤的诊治经过,并报告采用索拉非尼治疗其肺转移灶的经验。结果本例临床和影像学表现无特异性,患肾切除术后常规病理检查误诊为肾母细胞瘤,经免疫组化检查及联合病理会诊,确诊为肾原发性滑膜肉瘤(梭形细胞型)。采用依托泊苷、异环磷酰胺、顺铂化疗联合索拉非尼靶向药物治疗其肺部转移灶,获得部分缓解,但索拉非尼单药治疗及免疫治疗效果不佳。疾病控制期为6个月,生存期11个月。结论肾原发性滑膜肉瘤诊断困难,对肿瘤标本进行免疫组化或分子学分析方可确诊。一旦发生转移,预后不佳,联合采用以高剂量异环磷酰胺为基础的化疗和索拉非尼靶向药物治疗可能获得一定疗效。

Objective To improve the diagnosis and therapy of primary renal synovial sarcoma.Methods The clinical data of a case of primary renal synovial sarcoma was reviewed and experience of treating lung metastasis of this disease by sorafenib was reported.Results The clinical and imaging manifestations of the case were unspecific.The case was histologically misdiagnosed as adult Wilm＇s tumor after nephrectomy.Through immunohistochemical studies,primary renal synovial sarcoma（monophasic spindle cell subtype） was finally diagnosed by several pathological centers.Adjuvant vepeside,ifosfamide and cisplatin chemotherapy and targeted therapy with sorafenib were carried out postoperatively,and partial relief was achieved.Chemotherapy was ceased 3 months later because of severe side effects,and then the disease progressed through sorafenib continued and followed with immunotherapy as substitution.The disease was under controlled for 6 months,and the patient died 11 months after diagnosis.Conclusions If the histopathology of the resected renal mass is suggested to be primary synovial sarcoma,appropriate immunohistochemical,genetic and molecular tests must be performed to reach a firm diagnosis.The outcome was poor if metastasis occurred,and high-dose ifosfamide-based chemotherapy in combination with targeted therapy with sorafenib may be an effective adjuvant modality to radical nephrectomy.