原发性肾脏滑膜肉瘤2例报告并文献复习

目的探讨原发性肾脏滑膜肉瘤的诊断和治疗。方法对2例患原发性肾脏滑膜肉瘤的患者的临床资料进行总结，检索Pubmed及中国生物医学数据库。肾脏原发性滑膜肉瘤的相关文献。结果患者1术后病理报告：肾脏滑膜肉瘤，侵及肾皮质、髓质及肾周围脂肪组织。免疫组化结果：Vim、CK7、广谱CK、低分子CK、EMA、CD99、Bcb2均阳性，Des阴性。患者2术后病理报告：肾脏滑膜肉瘤，血管内见有瘤栓。免疫组化结果：Vim阳性、CK部分细胞、Des部分细胞、CD99部分细胞阳性，Myodl、CD34、Melanoma、CD68阴性。患者1术后7个月，患者2术后10个月均死于癌转移。检索相关文献显示有随访的33例中死亡11例，转移或复发14例。结论原发性肾脏滑膜肉瘤是一种高度恶性的罕见肿瘤，预后不良，确诊依赖病理检查、免疫组织化学。

Primary renal synovial sarcoma (report of 2 cases and literature review)

Objective To investigate the diagnosis and treatment of primary renal synovial sarcoma. Methods The data of 2 patients with primary renal synovial sarcoma were retrospectively analyzed, and the related literature in Pubmed and CBMdisc was retrieved. Results The diagnosis of primary renal synovial sarcoma was confirmed by postoperative pathology. Immunohistochemical staining showed that for the first patient, the markers Vim, CK7, CK （Pan）, CK （Low MW）, EMA, CD99 and Bcl-2 were positive in the tumor cells, while Des was negative. For another patient, the marker Vim was positive, CK, Des and CD99 were partial positive, while Myodl, CD34, Melanoma and CD68 were negative. Both cases died of tumor metastasis 7 or 10 months after the operation. Retrieved literature showed that of the 33 cases followed up, 11 cases died and 14 cases had tumor metastasis or recurrence. Conclusions Primary renal synovial sarcoma is a highly malignant and rarely seen tumor with poor prognosis. Its diagnosis depends on pathological and immunohistochemical examinations.