肾脏混合性上皮间质瘤二例报告及文献复习

Mixed epithelial and stromal tumor of the kidney (report of 2 cases and review of the literature)

目的探讨肾脏混合性上皮间质瘤的临床特征及诊治方法。方法报告2例肾脏混合性上皮间质瘤患者资料。2例均为女性,年龄分别为17及21岁。例1临床表现为无痛性全程肉眼血尿。B超、CT及MRI示右肾下极至肾门处一直径约8.0cm的囊性肿物,可见较多分隔。例2体检发现右上腹肿物。B超示右肝内大片强回声,肝内有多个小的无回声区,术前误诊为肝血管瘤,边界尚清。检索Medline及国内CBM数据库中相关文献,共报道51例,进行复习。结果2例均行手术完整切除肿瘤及同侧肾脏,大体观肿瘤由多发囊性和实性区域构成,镜下肿瘤由包含肾小管的梭形细胞间质和囊肿构成。免疫组化上皮抗体、vimentin、SMA及desmin等为阳性。2例术后分别随访22及60个月未见复发及转移。结论肾脏混合性上皮间质瘤临床上罕见,以女性多见,无特征性临床表现,生物学行为大部分为良性,治疗以单纯手术切除为主,恶性者尚无明确有效的治疗方案。

Objective To study the clinical and pathological features of mixed epithelial and stromal tumor of the kidney （ MESTK）, and its diagnosis and treatment. Methods Two cases of MESTK （both female; 17 and 21 years,respectively） were reported. The first case presented with a painless mass in the right upper abdomen. B-ultrasound showed large patches of strong echo with multiple small echoless,well- defined regions in the right liver. The other case presented with painless full-course gross hematuria. B-ultra sound, CT and MRI revealed a cystic mass of 8.0 cm in diameter with more compartments between the lower pole and hilus of the right kidney. The MEDLINE and CBM databases were searched for related articles. A total of 51 cases of MESTK were identified,and their clinical data were reviewed. Results Both tumors were resected completely with the ipsilateral kidney. The tumors consisted of multiple cystic and solid areas on gross examination, and both epithelial and stromal components were seen microscopically,the latter consisting of spindle shaped stromal cells. The epithelial antibody,vimentin,SMA and desmin were positive in immunohistochemical examination. There was no evidence of recurrence and metastasis during the follow-up of 60 and 22 months,respectively,in each case. Conclusions MESTK is a rare tumor which predominantly affects woman with benign biological behavior and no specific clinical feature in most cases. Surgery is the main therapy for MESTK and there is no confirmed effective therapy for malignant ones.