肾黏液管状及梭形细胞癌一例报告并文献复习

Renal mucinous tubular and spindle cell carcinoma：one case report and literature review

目的 提高对肾黏液管状及梭形细胞癌（mucinous tubular and spindle cell carcinoma,MTSCCa）的认识和诊治水平.方法 回顾性分析l例肾MTSCCa患者的病例资料并复习文献.患者女,38岁.2012年10月5日以上腹饱胀不适1个月余、血尿10d为主诉入院.查体：右侧肋弓下缘可触及一类圆形肿物,直径约7cm,质硬,无压痛,活动度尚可.B超检查：右肾上极混杂回声占位,11.0 cm ×7.5 cm,肿瘤及周边可见明显血流信号.CT检查：右肾上极内低密度影,累及右肾静脉,局部可见充盈缺损,下腔静脉内亦可见低密度影,增强后有不均匀延迟强化.双肾MRI检查：右肾上极巨大软组织肿块,最大截面积10.3 cm× 10.1 cm,T1WI稍低信号,T2WI稍高信号,增强后不均匀延迟强化.术前诊断：右肾透明细胞癌伴右肾静脉及下腔静脉瘤栓.全麻下行根治性右肾切除＋下腔静脉瘤栓取出术.术中见右肾巨大肿瘤,约10 cm×20 cm,右肾静脉和下腔静脉内瘤栓形成,下腔静脉内瘤栓长约8 cm.结果 手术顺利完成.术后病理检查：镜下可见大量梭形细胞成分交织束状排列,核分裂象易见.免疫组化染色检查：波形蛋白（±）,突触素、神经特异性烯醇化酶、CD99（＋）,Ki-67（70％＋）.TNM分期：pT3cN0M0期.Robson分期：Ⅲ期.病理诊断为高级别肾MTSCCa.术后1个月行白细胞介素-2（200万U）联合干扰素α-2a（500万U）免疫治疗28 d.治疗期间患者出现发热等类感冒样症状,每周复查血常规、肝肾功能无明显异常.术后2个月死于肺栓塞、肝肺多发转移.结论 MTSCCa是罕见的肾细胞癌病理类型,好发于女性,高级别病理类型预后极差.

Objective To improve the diagnosis and treatment of renal mucinous spindle cell carcinoma,and the knowledge of the type of kidney cancer.Methods The clinical date of 1 case of renal MTSCCa was reported.The case of patient was a 38-year-old women with abdominal discomfort and hematuresis.Ultrasound tip：the upper pole of the right kidney mixed echo placeholder,tumor and the surrounding is obvious blood flow signal.CT revealed a low density shadow in the upper pole of the right kidney,involving the right renal vein,partial filling defect,visible in the inferior vena cava are visible low density shadow,uneven enhancement after the reinforcement,and has delayed reinforcement.MRI shows：the upper pole of the right kidney huge soft tissue mass,T1WI shows low signal,T2WI show slightly higher signal,uneven enhancement and have delayed reinforcement.Preoperative diagnosis：right renal clear cell carcinoma with right renal vein and inferior vena cava tumor emboli.With kidney cancer radical resection and take out inferior vena cava tumor emboli,in the operation right kidney tumor,about 10 cm × 20 cm,right renal vein and inferior vena cava tumor emboli in formation,inferior vena cava tumor emboli within about 8 cm,the clinical and pathologic feature was analyzed and discussed with litcrature review.Results The operation smoothly.Pathological findings suggested higher grade mucinous tubular and spindle cell carcinoma;microscopically a large number of ingredients mixed bundle arrangement of spindle cells,nuclear fission as easy to see.Immunohistochemical staining：Vimentin ±,Syn ＋,NSE ＋,CD99 ＋,Ki-67 ＋ 70%.According to the 2010 AJCC for kidney cancer TNM staging,the pathological pT3c N0M0.According to the Robson stage]].The patient reserved interferonα-2a associate with interleukin-2 theraph after a month.During treatment in patients with fever and other flu symptoms,weekly review of routine blood,liver and kidney function has no obvious abnormalities.The patient died of pulmonary embolism,liver,lung multiple metastases two months later.Conclusions Renal mucinous tubular and spindle cell carcinoma is a rare cell types in the kidney pathological classification,women in high incidence,higher pathological types,worse prognosis.