肾恶性孤立性纤维肿瘤1例并文献复习

Renal malignant solitary fibrous tumor:1 case report and literature review

目的探讨肾恶性孤立性纤维肿瘤(SFT)的临床表现、影像学特点、诊断与鉴别诊断、治疗及预后。方法回顾性分析1例罕见的肾恶性SFT患者的临床资料,结合相关文献分析该病的组织来源、病理学特征、鉴别诊断及治疗随访情况。结果该例患者术前诊断为右肾透明细胞癌,行后腹腔镜根治性肾切除术,术后病理为右肾低度恶性SFT,免疫组织化学提示CD34、BCL-2、CD68、CD99、vimentin阳性表达,Ki-67 5%阳性表达及SMA局灶弱阳性。随访4个月,未见肿瘤复发或转移。结论肾恶性SFT非常罕见,诊断与鉴别诊断依据术后病理学及免疫组织化学,主要治疗方式为根治性肾切除术,预后较好。

Objective To investigate the clinical manifestations,imaging features,diagnosis and differential diagnosis,treatment and prognosis of renal malignant solitary fibrous tumor（SFT）. Methods The clinical data in 1 case of rare renal malignant SFT were retrospectively analyzed. Referring to related literatures, the histological origin, pathological features, differential diagnosis, treatment and follow-up of renal malignant SFT were analyzed. Results The patient was preoperatively diagnosed as right renal clear cell carcinoma. Postoperative pathological examination diagnosed as low grade malignant SFT of right kidney. And immunohistocbemistry indicated CD34^＋ , BCL-2^＋ ,CD68^＋ , CD99^＋ , vimentin, Ki- 675% , SMA focal weakly positive. No recurrence or metas tasis occurred after 4-month follow-up period. Conclusion Malignant SFT of the kidney is very rare,its diagnosis and differential diagnosis depend on postoperative pathological and immunohistochemical examination. Radical nephrectomy is the main option for malignant SFT of the kidney with good prognosis.