乳头状肾细胞癌临床分析

目的：总结分析乳头状肾细胞癌的临床特点,提高其诊治水平.方法：回顾性分析2003～2009年收治的乳头状肾细胞癌的临床资料.并与同期53例肾透明细胞癌比较.结果：乳头状肾细胞癌组患者年龄57.3（47～78）岁,皆为男性,占同期肾细胞癌9.4%.就诊时3例无症状,2例出现肉眼血尿,1例双侧腰痛伴腹部包块.肿瘤平均最大径为6.6（2.6～16.0）cm,未见多中心病灶.TNM分期：T1a N0 M0 3例,T1b N0 M0 1例,T2 No M0 2例 病理分型I型3例,Ⅱ型3例 Fuhrman分级Ⅱ级2例,Ⅲ级4例.与肾透明细胞癌相比,乳头状肾细胞癌好发于男性,影像学检查不具备恶性肿瘤特征,确诊有赖于病理和免疫组织化学检查.临床分期皆为I期或Ⅱ期.就诊时无一例出现远处转移.结论：乳头状肾细胞癌在临床表现上与肾透明细胞癌相似,但在影像学表现、病理形态及生物学行为上均与肾透明细胞癌有所不同.根治性肾切除术是目前首选治疗方式.靶向治疗有可能成为转移性乳头状肾细胞癌治疗的新方向.     

透明细胞乳头状肾细胞癌临床病理特征与预后分析

目的 探讨透明细胞乳头状肾细胞癌(CCPRCC)的临床病理特征和预后情况。方法 回顾性分析2011年6月—2021年10月于南京大学医学院附属金陵医院泌尿外科治疗的40例CCPRCC患者的临床、病理及随访资料。比较其与我院同时期40例透明细胞肾细胞癌(CCRCC)和19例乳头状肾细胞癌(PRCC)患者的预后情况。生存分析采用log-rank检验并绘制Kaplan-Meier生存曲线。结果 40例患者中，男性28例，女性12例；年龄31～84岁；38例为单侧，2例为双侧肿瘤；3例呈多灶性病变。所有患者均行手术治疗。肿块最大径3.0～95.0 mm,平均(27.6±18.1)mm。病理分级均为Fuhrman 1～2级。免疫组织化学检测CK7、CA-IX阳性。患者术后随访5～129个月，中位随访时间56个月：1例骨转移后死亡，2例同侧复发，1例罹患原发食管癌。CCPRCC患者生存预后明显好于CCRCC(P<0.001)和PRCC(P=0.005)患者，而CCRCC与PRCC患者预后差异无统计学意义(P=0.93)。结论 CCPRCC恶性程度低，确诊依靠特征性的病理学与免疫组化特点，手术...     

肾细胞癌病理亚型相关的二次原发肿瘤

作者评价肾细胞癌组织病理亚型——透明细胞癌、乳头状癌和嫌色细胞癌罹患二次肿瘤的风险。回顾性分析1970年至2000年因散发肾细胞癌行肾切除的2722例患者的病例资料，所有病理标本都由一位泌尿外科病理医师进行分型，二次原发肿瘤与肾细胞癌病理亚型的关系通过X^2检验和Fisher精确检验评价。结果：所有患者中，透明细胞癌2188例（80．4％），乳头状癌378例（13．9％），嫌色细胞癌128例（4．7％）。乳头状肾细胞癌患者比透明细胞癌患者更容易患结肠癌（P=0．041）、前列腺癌（P=0．003）和其他肿瘤（P＜0．001）。     

基于SEER数据库的肾透明细胞乳头状肾细胞癌患者临床特征及生存分析

目的 评估临床罕见的肾透明细胞乳头状肾细胞癌患者临床特征并进行生存分析。方法 获取2016—2019年SEER数据库中的肾透明细胞乳头状肾细胞癌患者的临床数据并进行临床特征描述，利用Kaplan-Meier法进行生存分析。结果 在SEER数据库中，共检索出191例临床资料完整且经组织学确诊的肾透明细胞乳头状肾细胞癌患者，其中男性112例(58.7%),女性79例(41.3%);组织学分级1～2级136例(71.2%),3～4级19例(10.0%);临床分期T1期174例(91.1%),T2～T3期17例(8.9%)。1例患者发生远处转移(肺转移合并淋巴结浸润和静脉瘤栓),2例发生静脉瘤栓。接受手术治疗181例(94.8%),根治性肾切除术和肾部分切除术是主要的手术类型，术后复发导致死亡1例，术后因其他原因死亡4例。整体患者第12、24个月生存率分别为98.5%、97.4%。结论 肾透明细胞乳头状肾细胞癌患者具有较低的临床分期和组织学分级，极少发生肿瘤进展和远处转移，具有良好的预后和极低的疾病特异性死亡率，行根治性肾切除术和肾部分切除术具有显著的治疗效果。     

乳头状肾细胞癌的诊断和治疗

目的；提高乳头状肾细胞癌的诊治水平。方法：8例均作影像学检查，IVU示患侧肾脏外形增大；CT示患侧肾脏有类圆形，界限清楚，中心有肿瘤坏死引起的代密度灶。8例患者均接受肾癌根治术，其中2例经腹腔途径，6例经11肋间切口。结果：随访4-60个月。除1例于术后13个月死于颅内转移外，其余均生存。结论：乳头状肾细胞癌是较罕见的肾细胞肿瘤，其形态学和遗传学与非乳头状肾细胞癌有明显不同；其确诊有赖于影像学检查，典型病理检查及分子遗传学特征分析；治疗仍以手术为主。     

乳头状肾细胞癌的临床病理表现

目的 探讨乳头状肾细胞癌的临床病理表现,提高对此类型肾癌的认识。方法 回顾性分析9例乳头状肾细胞癌患者临床病理资料并进行随访。结果 9例乳头状肾细胞癌占同肾癌的1.9％,平均发病年龄52岁。无自觉症状者5例,血尿4例。7例行肾癌根治术,1例行肾输尿管全长切除术,1例保留肾单位的肿瘤切除术。肿瘤最大径4－15cm,多位于肾皮质,淡黄色到金黄色,单灶或多灶性结节性长。均以乳头或乳头管状结构为主,乳头轴心可见泡沫细胞。9例均有细胞角蛋白CK7表达。Fuhrman病理分级G13例、G24例、 G32例。TNM病理分期pT27例、pT3a1例、pT3bN1V1b1例。随访7例无瘤生存36－134个月,1例G2pT3b者无瘤生存11个月,1例G3pT3a者13个月后死于肾癌转移。结论 乳头状肾细胞癌是少见的肾癌,表现为低分期,预后好。     

MR在肾肿瘤诊断中的应用

目的探讨MR在肾肿瘤临床诊断中的价值。方法回顾分析24例经病理证实的肾肿瘤患者临床资料,病理类型包括透明细胞性肾癌20例、乳头状肾细胞癌2例及血管平滑肌瘤2例。所有患者均行T1wI、T2WI、STIR序列检查,同时做MR动态增强扫描。结果透明细胞癌20例,T1wI均匀等信号7例,混杂信号10例,均匀低信号3例;T2WI均匀略高信号9例,明显高信号1例,混杂信号10例;显示假包膜者10例;肾窦侵犯3例,肾周侵犯4例,下腔静脉癌栓1例,腹膜后或肾门淋巴结肿大3例,腰大肌转移1例。注入造影剂后,7例病灶均匀强化,13例不均匀强化。乳头状肾细胞癌2例,T2wI低信号,增强后表现为持续轻度强化。血管平滑肌瘤2例,T1wI上的明显的高信号,T2WI为中等信号,无假包膜,脂肪可被明显抑制,无明显强化。结论MR对于肾肿瘤的术前检出、诊断有较高的准确率,具有重要的临床应用价值。     

肾脏黏液性管状及梭形细胞癌的临床病理观察

目的探讨肾脏黏液性管状及梭形细胞癌（mucinous tubular and spindle cell carcinoma,MTSCCa）的临床病理特点、免疫表型及鉴别诊断要点,并复习相关文献对其组织起源作一探讨。方法对1例肾脏黏液性小管状和梭形细胞癌进行临床病理学特征及免疫组化的分析。结果患者女性,56岁,无特异临床症状,体检发现右肾占位。巨检发现肾上极实质内一灰白界清结节,直径7cm,切面均匀。镜下见瘤细胞主要呈两种结构：由单层立方细胞排列成的细管状结构及类似于平滑肌瘤的梭形细胞结构,局部可见透明细胞及乳头样结构,立方状细胞及梭形细胞均形态温和。背景中见大量黏液,并可见淋巴细胞、浆细胞及泡沫细胞浸润。免疫组化：CK7、CK18、Vim阳性表达;SMA、HMB45、SYN均阴性。结论MTSCCa是一种少见的低级别肿瘤,预后较好,可能来源于远端肾单位。     

肾透明细胞癌胆囊转移2例报告并文献复习

目的提高对肾透明细胞癌胆囊转移的认识,探讨其临床及病理特点。方法报告2例经本院收治的肾透明细胞癌胆囊转移患者的临床资料,回顾性分析其临床特征以及影像学表现。2例均为男性,年龄分别为56岁及61岁。患者均无特征性临床表现。其中1例患者行腹腔镜根治性肾切除术后6个月复查B超发现胆囊腔内肿物增大,遂在全麻下行腹腔镜胆囊切除术。另1例发现肾脏及胆囊病变后同期在全麻下行腹腔镜根治性肾切除术及胆囊切除术。检索Pubmed和CBM数据库,对该疾病相关文献进行复习。结果2例术后病理诊断为肾透明细胞癌胆囊转移。术后随访3个月及25个月未见肿瘤复发。结论肾透明细胞癌胆囊转移临床罕见,对于肾癌患者或既往有肾癌病史的患者,应该关注其胆囊上存在的病变性质,PET／CT对诊断该病有一定帮助,同时比较原发灶和转移灶二者病理的异同能更准确地进行病理诊断。     

RASA1在肾透明细胞癌中的表达及临床意义

目的 基于肾透明细胞癌展开研究,明确肾癌组织中RAS p21蛋白激活因子1(RASA1)的表达水平,并探究其与临床病理特征及预后之间的相关性。方法 选取2010年1月1日至2018年12月31日在新疆医科大学第一附属医院进行手术切除的肾透明细胞癌患者,共91例。对其肾癌组织样本中RASA1的表达进行免疫组化检测,并分析其表达量与肾透明细胞癌患者临床病理特征及预后的关联性。结果 91例肾透明细胞癌患者中,T_1～2期患者共51例,T_3～4期患者共40例,临床病理资料统计分析表明,RASA1的表达水平与患者的T分期、M分期相关(P <0.05),T、M分期越高,则RASA1表达水平越低。RASA1表达与患者性别、年龄、民族、肿瘤大小、N分期、淋巴脉管是否受侵及病理分级无相关性(P>0.05)。此外,肾透明细胞癌患者的生存情况也与RASA1的表达水平密切相关,呈正相关。结论 RASA1的表达水平既与肾透明细胞癌的T、M分期相关,也与患者的预后相关。     

Clear cell papillary cystadenoma of the epididymis and mesosalpinx: immunohistochemical differentiation from metastatic clear cell renal cell carcinoma

Clear cell papillary cystadenoma is a rare epithelial tumor of the epididymis, which may present as an isolated lesion or as a component of von Hippel-Lindau disease (VHLD). Recently, tumors have also been described in the female genital tract with similar histology. Recognition of clear cell papillary cystadenoma is critical because of its association with VHLD and its potential diagnostic confusion with metastatic renal cell carcinoma because of a shared architecture and clear cells. In this study, we report on the immunohistochemical differentiation of 5 clear cell papillary cystadenomas, 3 of the epididymis and 2 of the mesosalpinx, from metastatic renal cell carcinoma. In 2 cases, there was a history of renal cell carcinoma in the setting of VHLD; and in 1 of these cases, an epididymal papillary cystadenoma was initially considered to be metastatic renal cell carcinoma. Immunohistochemically, tumor cells were moderately intensely positive for cytokeratin AE1/AE3 and epithelial membrane antigen, strongly positive for CK7 and negative for CK20 and RCC. Four of 5 cases were negative for CD10. This staining profile contrasts with that reported for clear cell renal cell carcinomas, which are typically negative for CK7 and immunoreactive for renal cell carcinoma (RCC) and CD10. Our findings indicate that, in cases where there is uncertainty about the histologic diagnosis of clear cell papillary cystadenoma, the above immunohistochemical panel helps to rule out metastatic renal cell carcinoma.     

Expression of alpha-methylacyl-CoA racemase in papillary renal cell carcinoma

Alpha-methylacyl-CoA racemase (AMACR) was first discovered by using cDNA microarray technology as a molecular marker for prostate cancer. Our recent microarray analysis of renal cell carcinomas showed a significant increase of AMACR mRNA levels in papillary renal cell carcinomas, but not in other subtypes. To investigate the value of this marker in pathologic diagnosis, we analyzed AMACR mRNA levels in cDNA microarrays from 70 kidney tumors. Furthermore, we evaluated the AMACR expression in 165 kidney tumors on tissue microarrays and 51 papillary carcinomas of other organs by immunohistochemistry. AMACR mRNA was significantly overexpressed in 7 of 8 papillary renal cell carcinomas with an average of 5.2-fold increase, and only in 2 of 62 nonpapillary kidney tumors. Immunohistochemistry demonstrated strong AMACR positivity in all cases of papillary renal cell carcinomas (41 of 41, 100%), including 6 metastatic papillary renal cell carcinomas, but only focal or weak reactivity in the minority (18 of 124, 15%) of other renal tumors including 13 of 52 clear cell renal cell carcinomas, 3 of 20 oncocytomas, and 2 of 17 urothelial carcinomas. All chromophobe (0 of 18) and sarcomatoid components of renal cell carcinomas (0 of 15) were negative for AMACR. Weak or focal AMACR immunoreactivity was detected in only 4 of 51 (8%) papillary carcinomas arising in other organs (2 of 14 thyroid, 2 of 13 lung, 0 of 6 breast, 0 of 6 endometrium, 0 of 6 ovary, and 0 of 6 pancreas). Using a combination of cDNA microarrays, tissue microarrays, and immunohistochemistry, we identified AMACR as a marker for papillary renal cell carcinoma, which could be valuable in subclassification of renal cell carcinomas and in the differential diagnosis of a metastatic papillary carcinoma.     

Imaging characteristics of papillary renal cell carcinoma by computed tomography scan and magnetic resonance imaging

AIM: To analyse the differences in the patterns between clear and papillary renal cell carcinomas using magnetic resonance imaging (MRI) and dual-phase helical computed tomography (CT). METHODS: We examined seven patients with papillary renal cell carcinoma, and six with clear cell carcinoma. The highest attenuation value of tumors in the corticomedullary phase (CMP) and the excretory phase (EP) was measured using the observer-defined region of interest (ROI). MRI consisted of T1-weighted and T2-weighted spin-echo imaging. RESULTS: All five tumors except for one with papillary renal cell carcinoma showed homogenous hypointensity, but all six tumors with clear cell carcinoma showed heterogeneous hyperintensity on their T2-weighted images. In the CMP, the mean CT numbers of the papillary renal cell carcinomas were significantly lower than those of the clear cell carcinomas. The mean enhancement of the papillary renal cell carcinomas in the CMP and the EP was significantly lower than that of the clear renal cell carcinomas. The mean CT numbers of the clear cell carcinomas in the CMP were markedly increased from those on the unenhanced CT; those in the EP were decreased gradually. But the mean CT numbers of the papillary renal cell carcinomas in the EP were still slightly more increased than those in the CMP. The enhancement patterns of the papillary renal cell carcinomas in the CMP and the EP were homogenous, but those of the clear cell carcinomas were heterogeneous. CONCLUSIONS: We can speculate the differential diagnosis from clear to papillary renal cell carcinoma using MRI and dual-phase helical CT.     

晚期肾癌索拉非尼治疗后形态学改变二例报告

目的 探讨晚期肾癌患者索拉非尼治疗后形态学及肾癌相关蛋白表达改变的特点.方法 晚期肾癌索拉非尼治疗后的组织2例,行HE染色病理学检查,应用国内常用组织学分级系统评价治疗反应,免疫组织化学方法检测原发性肿瘤组织与复发或转移灶索拉非尼治疗后的免疫表型改变. 结果 2例患者标本病理类型与治疗前无改变,均出现不同程度的治疗反应,表现为肿瘤细胞轻度退行性改变,肿瘤组织可见明显坏死及间质纤维化.免疫组织化学分析结果显示索拉非尼治疗前后肾癌相关抗原Vimentin、AE1/AE3、CK7、CK8、CK18及CD10蛋白表达无明显变化,索拉非尼治疗后肾癌组织中血管内皮生长因子(VEGF)、VEGF受体-2(VEGFR2)、p53及Ki-67蛋白表达增强,而Bcl-2蛋白表达降低. 结论 索拉非尼治疗后可引起转移癌或复发肿瘤与原发肿瘤之间的形态学差异,以瘤细胞退行性改变及间质纤维化为主要表现,但转移性肾癌病理组织学类型无改变.VEGF、VEGFR2等表达的变化可能与索拉非尼疗效相关.     

Renal cell carcinoma in the solitary kidney: an analysis of complications and outcome after nephron sparing surgery

PURPOSE: We evaluated surgical techniques, pathological features and extended outcomes in patients with renal cell carcinoma in a solitary kidney treated with surgical excision. MATERIALS AND METHODS: Between 1970 and 1998, 76 patients underwent nephron sparing surgery for sporadic renal cell carcinoma in a solitary kidney, including 63 with tissue specimens available for pathological review who comprised the cohort. Six (9.5%) patients had a congenitally absent kidney and 57 (90.5%) had previously undergone contralateral nephrectomy for renal cell carcinoma. The clinical and pathological features examined were patient age at nephron sparing surgery, sex, type of nephron sparing surgery (enucleation, partial nephrectomy or ex vivo resection), tumor size, nuclear grade, histological subtype and 1997 tumor stage. Overall cancer specific, local recurrence-free and metastasis-free survival as well as early (within 30 days of nephron sparing surgery) and late (30 days to 1 year after nephron sparing surgery) complications were assessed. Univariate and multivariate analyses were done to test for the associations of clinical and pathological features with outcome. RESULTS: Most patients were treated with enucleation (36.5%), standard partial nephrectomy (38.1%) or the 2 procedures (11.1%) and in 8 (12.7%) ex vivo tumor resection was done. The renal cell carcinoma histological subtypes were clear cell in 82.5% of cases, papillary in 15.9% and chromophobe in 1.6%. Grade was 1 to 3 in 10 (15.9%), 42 (66.7%) and 10 (15.9%) tumors, respectively. At 5 and 10 years the overall survival rate was 74.7% and 45.8%, the cancer specific survival rate was 80.7% and 63.7%, the local recurrence-free survival rate was 89.2% and 80.3%, and the metastasis-free survival rate was 69% and 50.4%, respectively. Tumor stage and nuclear grade were significantly associated with death from any cause, death from renal cell carcinoma and distant metastases on multivariate analysis. Notably no patient with papillary or chromophobe renal cell carcinoma died of renal cell carcinoma, or had recurrence or metastasis. The type of nephron sparing surgery was not significantly associated with outcome, although there were too few patients with recurrence to assess the association of the type of nephron sparing surgery with local recurrence. The most common early complication was acute renal failure in 12.7% of cases, while the most common late complications were proteinuria in 15.9% and renal insufficiency in 12.7%. CONCLUSIONS: The 1997 tumor stage and nuclear grade were significant predictors of death from any cause, death from renal cell carcinoma and distant metastases in patients treated with nephron sparing surgery for renal cell carcinoma involving a solitary kidney. Nephron sparing surgery in a solitary kidney can be performed safely and with minimal morbidity.     

Renal cell carcinomas in children and young adults: increased incidence of papillary architecture and unique subtypes.

Renal cell carcinomas in children and young adults are rare, and the pathologic features of these tumors have not been well described. We reviewed 24 renal cell carcinomas in children and young adults ages 6 to 29 years, 14 of whom were younger than 18 years of age. Fourteen were female. In 19 (79%) of 24 cases, the tumor met histologic criteria for papillary renal cell carcinoma, with at least 50% papillary architecture. Four of the remaining five cases were typical clear cell tumors in patients known to have von Hippel Lindau syndrome, and one case was of chromophobe type. In the papillary tumors, calcifications, high nuclear grade, extracapsular extension (American Joint Commission on Cancer stage T3), and lymph node metastases were common. Among these papillary tumors, four distinct histologic patterns could be identified. Collecting duct-like tumors (two cases) involved the large collecting ducts, were multifocal and predominantly papillary, and had focal tubular and solid areas. These tumors were reactive for epithelial membrane antigen (EMA) and keratins, including CK7, but negative for Ulex europeaus and high molecular weight keratin 34BE12. Voluminous cell tumors (four cases) were composed of cells with extremely voluminous clear cytoplasm and, although predominantly papillary, had areas that also resembled clear cell tumors. These tumors were reactive for keratins AE1/AE3 but were otherwise negative for all other keratins, EMA, and U. europeaus. One of these tumors showed an X;7 translocation. Adult type tumors (12 cases) resembled papillary tumors of adults. These tumors were reactive for EMA and keratins, including CK7, and all but one were negative for U. europeaus and keratin 34BE12. This last case had trisomies of chromosomes 7, 16, 17, and 20. The final neuroendocrinelike case was multifocal, organoid, and composed of nests of small cells in a neuroendocrinelike pattern. Three of 13 patients were alive with disease at last follow-up, and three additional patients died of disease, all within 2 years. Progression was highly associated with lymph node involvement at the time of resection. We conclude that the clinicopathologic features of renal cell carcinomas in children and young adults differ from those arising in older adults. These tumors are characteristically high-grade, high-stage, papillary tumors with numerous calcifications, and several subtypes can be identified based on histologic, immunohistochemical, and cytogenetic features. Some subtypes appear to be unique to this age group.     

索拉非尼治疗具有肉瘤样分化的转移性肾癌的疗效观察

目的 分析索拉非尼治疗具有肉瘤样分化的转移性肾癌的疗效.方法 已行肾脏原发肿瘤切除的转移性肾细胞癌患者14例,病理证实原发肿瘤中含有肉瘤样分化成分.平均年龄61(45～77)岁.肾透明细胞癌伴肉瘤样分化8例,乳头状癌伴肉瘤样分化2例,单纯肉瘤样癌4例,肾原发病灶中肉瘤样成分比例为20%～100%.肿瘤转移部位分别为肺、淋巴结、肾上腺、肝或骨.采用索拉非尼400 mg或600 mg,2次/d治疗.采用Kendall相关检验和Pearson相关检验分别检测肉瘤样成分比例与治疗客观反应及中位无疾病进展时间(PFS)的相关性.中位治疗时间8(3～19)个月.结果 部分缓解(PR)2例,其转移病灶均位于腹膜后及纵隔淋巴结,肉瘤样成分比例分别为100%和20%;疾病稳定(SD)7例,转移部位包括肺、淋巴结、肾上腺、骨和肝;疾病进展(PD)5例,总疾病控制率为64%.随访至2009年7月,出现PD 9例,PFS 6(0～19)个月.肉瘤样成分比例与治疗的客观反应及PFS均无相关性(P=0.247,P=0.554).结论 索拉非尼常规剂量治疗具有肉瘤样分化的转移性肾细胞癌有一定疗效,但客观有效率及PFS与肉瘤样成分的比例无明显相关性.     

肾嫌色细胞癌临床特征分析

目的 探讨肾嫌色细胞癌的临床特点并分析其预后.方法 对1998年1月至2008年1月期间收治的29例肾嫌色细胞癌患者的临床资料包括临床表现、影像学检查、治疗方法、Ptnm分期和生存期随访等,进行回顾性分析.结果 所有29例患者均缺乏典型的临床症状,术前影像学检查亦未发现特异性表现.23例患者行根治性肾切除术,6例行保留肾单位手术(NSS),病理分期:Pt1n0m0 11例,Pt2n0m0 8例,pT3aN0M0 5例,Pt1nm0 3例,Pt2n1m0 2例.26例患者获得随访,随访24～144个月,平均90个月;3例死于心脑血管疾病,6例局部复发后4例再次手术,1例远处转移,21例无瘤生存.与同期肾透明细胞癌患者相比,累积5年生存率两者分别为83.9%和63.8%,差异无统计学意义(P＞0.05),累积10年生存率分别为77.9%和49.9%,差异具有统计学意义(P＜0.01).结论 肾嫌色细胞癌是一种少见的具有特殊形态的肾细胞癌病理亚型,主要通过病理学予以鉴别诊断,手术切除是治疗初发或复发的肾嫌色细胞癌的首选方法,其预后较好.

Abstract：

Objectives To explore the clinical, pathological features and prognosis of patients with chromophobe renal cell carcinoma. Methods From January 1998 to January 2008, clinical data of 29 patients with chromophobe renal cell carcinoma including clinical manifestations, imaging examinations,treatment models, pTNM stages and follow-up results, were summarized to investigate its features and prognosis. Results All cases had no obvious clinical and preoperative imaging presentation. There were 23 patients underwent radical nephrectomy, and 6 cases underwent nephron sparing surgery. Postoperative pathological findings confirmed the diagnosis of chromophobe renal cell carcinoma. Macroscopically, the cut surface of the tumors were generally beige in color. Histologically, it showed polygonal chromophobe cells and small round eosinophilic cells with eccentric hyaline degeneration. These tumor cells had a clear and sharp membrane, lightly stained abundant cytoplasm with a fine reticular translucent pattern and irregular nuclei. And a perinuclear halo was often seen in these cells. Histochemically, the tumor cells generally show a diffuse and strong reaction for CK-8 with a negative expression of Vimentin. The pTNM stages of the tumor were as follows, pT1N0M0 in 11 cases, pT2N0M0 in 8 cases, pT3aN0M0 in 5 cases, pT1N1M0 in 3 cases,pT2N1M0 in 2 cases. Twenty-six cases of patients were followed up (24 to 144 months, with an average of 90 months), 3 cases died of cardio-cerebrovascular disease, and local recurrence involved in 6 cases with reoperation in 4 cases, as well as distant metastasis in 1 case. Twenty-one cases survived with tumor-free.The statistical results indicated that the survival rates of the patients with chromophobe renal cell carcinoma in five years and ten years were 83.9%, 77.9%, respectively, compared with renal cell carcinoma of the same stage 63. 8% and 49. 9% at the same periods, and there is no difference in the survival rate of five years( P ＞ 0. 05 ) but significant difference in that of ten years ( P ＜ 0. 01 ). Conclusions Chromophobe renal cell carcinoma is a morphologically uncommon subtype of renal cell carcinoma with the good prognosis. Definite diagnosis depends on its typical pathological feature. Radical nephrectomy is the first choice for the treatment of chromophobe renal cell carcinoma.