肾混合性上皮间质肿瘤7例临床病理特征分析及鉴别诊断

Clinicopathological characteristics and differential diagnosis of mixed epithelial and stromal tumor of the kidney:report of seven cases

目的肾混合性上皮间质肿瘤的临床表现及影像学特点不典型,术前诊断困难,需病理确诊,本研究总结其临床病理特征及预后,以提高对此疾病的病理诊断及鉴别诊断。方法回顾性分析7例肾混合性上皮间质肿瘤,结合文献分析其临床病理特征、预后及鉴别诊断。结果7例肾混合性上皮间质肿瘤患者全部为女性,年龄为24~55岁,平均年龄45岁。肿瘤全部为单发,大体表现为多房囊性或囊实性。组织学形态特点为上皮组织和间质组织混合组成。免疫组化染色显示上皮细胞表达AE1/AE3阳性(6/6),间质细胞表达PR阳性(6/6),ER阳性(6/6),Actin阳性(2/5),Desmin阳性(6/6),SMA阳性(3/3),CD34阳性(0/4),S-100阳性(0/2),HMB45阳性(0/3),Ki-67增殖指数比较低。所有病例均接受手术治疗,其中行根治性肾切除术5例,行肾部分切除术2例。术后随访13~175个月,中位随访115个月,患者均未发现肿瘤复发与转移。结论肾混合性上皮间质肿瘤是非常少见的良性肿瘤,其诊断和治疗仍以手术切除为主,预后好。本病比较少见及报道病例不多,因此,还需要进一步了解其发病机制、临床行为、恶性潜能及治疗管理。

Objective To investigate the clinical manifestations,imaging changes,clinicopathological features,diagnosis and prognosis of mixed epithelial and stromal tumor of the kidney(MESTK).Methods The clinical and pathological features,prognosis and differential diagnosis of 7 patients with renal mixed epithelial-stromal tumor were analyzed retrospectively.Results The 7 patients with renal mixed epithelial-stromal tumor were all female,aged 24-55 years with a mean age of 45 years.The tumors were all single and were generally multilocular cystic or cystic solid.Histological features were the mixed composition of epithelial tissue and stromal tissue.Immunohistochemical staining showed in epithelial cells AE1/AE3 was positive(7/7);in stromal cells PR positive(7/7),ER positive(6/6),actin positive(2/5),desmin positive(6/6),SMA positive(3/3),CD34 negative(0/4),and S-100 negative(0/2)and HMB45 negative(0/3).All patients received surgical treatment,including radical nephrectomy in 5 cases and partial nephrectomy in 2 cases.Postoperative follow-up was 13-175 months,with a median follow-up of 115 months.No recurrence or metastasis was found in all patients.Conclusion Mixed epithelial-stromal tumor of the kidney is a very rare benign tumor.The diagnosis and treatment of mixed epithelial-stromal tumor of the kidney are mainly surgical resection,but a few cases may have recurrence and malignant transformation.Therefore,further understanding of its pathogenesis,clinical behavior,malignant potential,and treatment management is needed.