【摘要】：目的探討腎臟肉瘤樣癌的臨床表現(xiàn)、病理學(xué)特征、預(yù)后及治療。 方法回顧性分析本院2007年3月至2013年10月全部腎臟肉瘤樣癌41例,男31例,女10例。年齡34～83歲,平均年齡61歲。腰腹部酸脹或疼痛不適19例,無(wú)痛性肉眼血尿10例,乏力消瘦4例,發(fā)熱4例,尿路刺激癥狀3例,排尿不暢2例,體檢發(fā)現(xiàn)9例。腰腹部可及腫塊5例,腎區(qū)叩痛4例,無(wú)陽(yáng)性體征32例。41例患者術(shù)前均行B超檢查,其中33例同時(shí)行CT檢查,14例同時(shí)行MR檢查,腫瘤大小2.5×1.5cm～18.0×14.0cm,平均7.0×4.6cm。3例術(shù)前B超與MRA檢查顯示有腎靜脈癌栓,其中2例伴有下腔靜脈癌栓。38例行根治性腎切除術(shù)并行區(qū)域淋巴結(jié)清掃,2例行保留腎單位手術(shù),1例僅行腫瘤穿刺活檢。15例術(shù)后予IFN-α免疫治療,8例術(shù)后予舒尼替尼靶向治療。 結(jié)果本組41例患者術(shù)后病理示：腎細(xì)胞癌伴部分肉瘤樣癌22例,單純腎肉瘤樣癌9例,腎盂尿路上皮癌伴部分肉瘤樣癌6例,腎集合管癌伴部分肉瘤樣癌2例,腎盂鱗狀細(xì)胞癌伴部分肉瘤樣癌2例。TNM分期：Ⅰ期10例,Ⅱ期11例,Ⅲ期15例,Ⅳ期5例。25例行免疫組化檢查,結(jié)果顯示細(xì)胞角蛋白和波形蛋白均呈陽(yáng)性表達(dá)。獲得隨訪39例,28例死亡,11例生存,總生存時(shí)間1～61個(gè)月,中位生存期14.7個(gè)月。亞組中位生存期統(tǒng)計(jì)：TNM分期Ⅰ～Ⅱ期組18.1個(gè)月,Ⅲ～Ⅳ期組10.6個(gè)月；術(shù)后予IFN-α免疫治療組18.0個(gè)月；術(shù)后予生物靶向治療組16.2個(gè)月；組織學(xué)為單純腎肉瘤樣癌組11.5個(gè)月。 結(jié)論腎臟肉瘤樣癌是一種罕見(jiàn)的腎臟惡性腫瘤,惡性程度高。臨床癥狀明顯,影像學(xué)較難與普通腎癌相區(qū)分,確診主要依賴病理及免疫組化,治療以根治性腎切除為主,預(yù)后差,臨床分期及肉瘤樣成分含量是影響預(yù)后的重要標(biāo)準(zhǔn)。對(duì)于臨床上懷疑腎肉瘤樣癌的患者必須引起重視,盡早明確診斷,盡早行手術(shù)治療的同時(shí)可應(yīng)用化療或靶向藥物行輔助治療。
[Abstract]:Objective to investigate the clinical manifestations, pathological features, prognosis and treatment of renal sarcomatoid carcinoma. Methods from March 2007 to October 2013, 41 cases of renal sarcomatoid carcinoma were retrospectively analyzed, including 31 males and 10 females. The average age was 61 years. There were 19 cases of abdominal distention or pain, 10 cases of painless hematuria, 4 cases of fatigue and wasting, 4 cases of fever, 3 cases of urinary tract irritation, 2 cases of dysuria, and 9 cases of physical examination. Five cases of accessible lumps in waist and abdomen, 4 cases of knocking pain in renal area, 32 cases without positive signs and 41 cases were examined by B-ultrasound before operation. Among them, 33 cases underwent CT examination and 14 cases underwent Mr examination at the same time. The tumor size was 2.5 脳 1.5cm~18.0 脳 14.0 cm, with an average of 7.0 脳 4.6cm.3. B-ultrasonography and MRA showed renal vein tumor thrombus before operation. Among them, 2 cases were accompanied with inferior vena cava tumor thrombus. 38 cases were treated with radical nephrectomy and 2 cases with nephron-sparing surgery. One case received tumor biopsy only. 15 cases received IFN- 偽 immunotherapy and 8 cases received sunitinib targeted therapy after operation. Results the pathological findings of 41 patients were as follows: 22 cases of renal cell carcinoma with partial sarcomatoid carcinoma, 9 cases of simple renal sarcomatoid carcinoma, 6 cases of renal pelvis urothelial carcinoma with partial sarcomatoid carcinoma, 2 cases of renal collecting duct carcinoma with partial sarcomatoid carcinoma. TNM staging was performed in 2 cases of squamous cell carcinoma of renal pelvis with sarcomatoid carcinoma: 10 cases in stage 鈪，

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