肾集合管癌临床及病理分析

目的 分析肾集合管癌(collecting duct carcinoma,CDC)的临床及病理特点. 方法 1999年1月至2010年12月收治CDC患者11例,男6例,女5例.年龄22～67岁,平均55岁.主要症状为血尿、腰腹痛.实验室检查无阳性发现.CT检查示肿瘤直径2.1～8.5 cm,平均5.6 cm.肿瘤位于肾髓质或同时伴有肾皮质、肾盂浸润,边界不清,病变肾脏外形增大,但肾脏轮廓基本存在,增强后呈不均匀轻～中度强化. 结果 8例行根治性肾切除术,3例行姑息性肾切除术.肿瘤切面呈灰白色,浸润性生长;以腺管乳头状结构为主,部分肿瘤细胞呈靴钉状突向腺腔内,肿瘤间质纤维组织增生明显,有较多淋巴细胞及浆细胞浸润;免疫组化染色检查UEA-1、EMA、PNA、HMW-CK表达阳性,而CD10表达阴性.10例获得随访,随访时间0.3 ～8.0年,平均2.8年.随访期间死亡7例,平均生存期为12.5个月,2例无瘤生存分别9个月和8年,1例仍在化疗中,1例失访. 结论 CDC是一种非常少见的肾癌类型,确诊主要依据病理学检查,肿瘤恶性程度高,进展迅速,预后差.     

肾集合管癌8例临床病理分析

目的探究肾集合管癌的临床及病理特征。方法回顾性研究我院收治的8例肾集合管癌患者的临床、病理资料并进行随访。结果 8例患者均施行肾根治性切除术,术中见肿瘤直径5~12cm。肿瘤主要位于肾髓质,呈侵袭性生长,以腺管或腺管乳头状结构为主,部分表现为片状生长,间质内含较多淋巴细胞,并伴有集合管上皮的异型增生。免疫组化显示肿瘤细胞均表达CK7、CK19、上皮膜抗原(EMA)、34βE12、PNA,UEA-1、vimentin呈部分阳性表达,而HMB45和S-100为阴性。随访患者中4例患者发生转移,2例死于肾癌转移,2例带瘤生存。平均生存时间为12.8个月。结论肾集合管癌较罕见,恶性程度高,侵袭性强,预后差。根治性手术为主要治疗手段,术后靶向治疗可能改善患者预后,但仍需临床试验进一步验证。     

肾集合管癌的临床病理研究

肾集合管癌 (ｃｏｌｌｅｃｔｉｎｇｄｕｃｔｃａｒｃｉｎｏ ｍａｏｆｋｉｄｎｅｙ,ＣＤＣＫ)是一种少见的肾恶性肿瘤[1] 。此肿瘤的生物学行为和病理特点不同于常见的透明细胞癌而被ＷＨＯ分为独立的一类[2 ] 。我院诊治 10例 ,报道如下。1.资料和方法 :从 1989年 1月至1999年 6月 30日 ,我院共诊治肾癌 46 6例 ,其中ＣＤＣＫ10例 ,占 2 1%。男 6例 ,女 4例。平均年龄 5 0岁 (2 8～ 77岁 ) ,其中 37岁以下 5例。左肾 4例 ,右肾 5例 ,双肾 1例。因血尿而就诊的 7例 ,腰疼 2例 ,腹疼伴低热及肿块 1例。病程 1周到 1年不等 ,平均 4 8个月。经…     

肾集合管癌的临床特征分析

目的探讨肾集合管癌的临床病理特征及预后。方法回顾性研究2007年3月至2012年6月间收治的5例肾集合管癌患者的临床及病理资料并进行随访。结果5例肾集合管癌占同期肾癌的0．74％,男3例,女2例,平均年龄61岁。左肾4例,右肾1例。主要症状为血尿、腰腹痛。其中4例B超示肾占位性病变,1例示肾盂占位性病变。CT增强后强化不均匀。其中1例入院时双肺多发结节,其余4例人院时均未发现转移。2例行开放性根治性肾切除术,3例行肾切除术,术后病理报告为肾集合管癌伴乳头状结构。1例免疫组化示CK7阳性、vimentin部分阳性、CK20阴性,其余4例均无免疫组化结果。3例分别于术后9、12和15个月死于肾癌转移,2例无瘤生存。3例平均生存时间12．3个月。结论肾集合管癌临床症状明显,侵袭力强,预后差。目前术前诊断主要依赖CT和B超检查;主要治疗方法为根治性肾切除术,术后靶向治疗可能改善患者预后,但仍需要临床试验进一步验证。     

肾集合管癌预后不良原因的初步分析

目的 初步分析肾集合管癌预后不良的原因,探讨改善集合管癌预后的方法.方法 回顾性分析2003年6月至2007年10月648例肾癌中7例集合管癌患者(1.08%)的临床、病理及随访资料;通过患者症状、TNM分期、综合治疗策略三个方面综合初步分析集合管癌预后不良的原因.结果 7例集合管癌患者,男性4名,女性3名,平均年龄58.3岁.因腰痛、血尿、腹部肿块就诊者5例,无症状肾癌2例.AJCC临床分期Ⅰ期2例,Ⅳ期5例.肿瘤平均体积8.2 cm×6.6 cm×5.8 cm,血管内癌栓形成5例,肾内转移形成卫星灶3例,肾门淋巴结转移4例,远处转移1例.5例行根治性肾切除术,2例行姑息性肾切除术,1例加行胰十二指肠切除术.7例术后均行免疫治疗,1例行辅助化疗.平均随访13.9个月,5例死亡患者平均生存时间11.8个月.结论 典型症状的出现提示预后不良,早期淋巴转移,根治性切除术对大部分患者难以达到根治效果,术后辅助治疗效果不明确是集合管癌预后不良的重要原因.依靠健康查体筛查可疑病例,早期发现,早期手术治疗是改善肾集合管癌预后的关键所在.     

肾集合管癌（附五例报告）

肾集合管癌少见，其生物学行为尚不十分清楚。我们在近４年间收治５例，男３例，女２例，年龄４１～６７岁。主要临床表现为血尿、消瘦、低热。４例行根治性肾切除术，３例分别经３个月，２、２．５年随访无复发，ｌ例术后１年２个月死于肺转移。１例因肾门、腔静脉旁淋巴结广泛受累仅作姑息性肾切除，术后２个月出现骨转移。病理所见：肿瘤灰白，位于髓质，镜检呈腺管乳头状结构，间质丰富，邻近集合管异型增生。免疫组化检查高分子细胞角蛋白，花生凝集素及上皮细胞膜抗原均阳性。结合文献对集合管癌的若干诊断和治疗问题进行讨论。     

肾集合管癌2例报告及文献复习

目的：探讨肾集合管癌（CDC）的临床、病理特点及诊治方法。方法：回顾性分析2例CDC患者的临床资料。2例分别为男性44岁和女性60岁,分别因间断右腰痛1个月和间断无痛肉眼血尿1周人院。彩超和CT检查发现。肾门部位肿物,与周围肾组织界限不清晰,CT增强扫描肿物仅轻微强化。2例均于全麻下行肾根治性切除术。结果：大体标本见肿物位于。肾门部位,无包膜,呈浸润性生长,与周围组织无明确分界。镜下见肿瘤细胞排列成不规则腺管或腺管乳头状结构,Fuhrman细胞核分级3～4级。免疫组化反应1例高分子量细胞角蛋白（CK）和花生凝集素（PNA）均呈阳性,另1例PNA阴性,但高分子量CK呈阳性。患者分别于术后8个月、13个月死亡。结论：CDC发病率低,临床罕见。CT对术前诊断可提供帮助,确定诊断依赖病理学检查。转移早、预后差是CDC的特征之一。     

肾集合管癌的临床病理特征及治疗（附5例报告）

目的：探讨肾集合管癌的临床病理特征,提高对。肾集合管癌的诊断和治疗水平。方法：回顾性分析5例肾集合管癌的临床资料：男4例,女1例,平均年龄54（42～65）岁。主要症状为腰腹痛、血尿及腹部包块和低热。4例患者行根治性肾癌切除术,1例行姑息性肾切除术。术后行病理学检查。结果：淋巴结转移3例,肾门脂肪转移2例;肿瘤位于肾髓质,侵袭性生长,以腺管或乳头状结构为主,特征性靴钉样细胞衬覆于管腔内面,伴有间质纤维反应,瘤旁集合管上皮细胞异型增生。AJCC临床分期Ⅱ期1例,Ⅲ期和Ⅳ期各2例。术后干扰素免疫治疗2例。随访5～18个月,5例患者平均生存10个月。结论：肾集合管癌具有特殊的临床病理特征,恶性程度高,预后差,其确诊依赖于病理学检查。早期发现、早期手术治疗仍是改善集合管癌预后的主要方法。     

肾集合管癌1例并文献复习

肾集合管癌是一种少见的恶性肿瘤。我院曾诊治1例，现报告分析如下。     

肾集合管癌的临床特点及预后(附4例报告)

目的：探讨肾集合管癌（Collecting duct carcinoma,CDC）的临床特点及预后．以提高对CDC的诊治水平。方法：回顾性研究4例CDC患者的临床特点及病理资料并进行随访。结果：4例CDC患者占同期肾癌的1．41％,全部为男性,年龄为51～62岁。主要症状为血尿、腰腹痛、乏力、消瘦及低热。其中1例术前诊断为肾结核,但术中探查发现合并输尿管多发结节,冷冻切片病理检查为输尿管癌,行患侧肾癌根治性切除加输球管全长及膀胱部分切除术,术后病理检查为CDC并输尿管移行细胞癌。2例术前发现双肺多发转移病灶,同时肾门、肾动脉与腹主动脉间多发肿大淋巴结,术中尽量切除可疑淋巴结,术后病理检查证实为淋巴结转移。另1例术再病理检查报告为CDC,部分为透明细胞癌。肿瘤TNM分期：2例为T2N2M1（Ⅳ期）,1例为T2N1Mn（Ⅲ期）,1例为T1N0M0（Ⅰ期）。4例患者术后均进行干扰素及IL-2治疗,2例有远处转移者同时行放疗。1例患者术后2个月因肝、肺、肋骨多发转移而死亡,余3例目前仍在随访中。结论：CDC临床症状明显,进展快,恶性程度高,顶后差。主要治疗方法为肾癌根治术,患者多于术后数月或几年内发生转移而死亡。     

Collecting duct carcinoma with acquired cystic disease of the kidney in a long-term hemodialysis patient

Abstract:   We report a very rare case of collecting or Bellini duct carcinoma (CDC) found in a 60-year-old male who had received hemodialysis therapy for 21 years. Screening with ultrasonography revealed a solid tumor originating from the cyst wall in the right kidney with acquired cystic disease of the kidney. Subsequent computed tomography (CT) and angiography could not detect another renal tumor. Right radical nephrectomy was performed. The tumor detected preoperatively was composed of papillary renal cell tumor (RCC) and multiple clear cell carcinoma, pathologically. In addition to the tumors, CDC was revealed in the central medulla with the involvement of regional lymph nodes. Three months later, left nephrectomy was performed because left RCC was suspected during CT. The histological diagnosis was multiple clear cell carcinomas. Peritonitis carcinomatosa appeared and the patient died 13 months later.     

The collecting duct carcinoma of the kidney: a cytogenetical study

OBJECTIVES: The Heidelberg classification of renal tumours identifies five histotypes of renal cancer, underlining for two of them (conventional and papillary renal cancers) a strict relation between the morphological aspect and the complement of alterations evidenced by the cytogenetic analysis of the neoplastic karyotype. Due to its low incidence, the collecting duct carcinoma (CDC) has not yet been characterized from a cytogenetic point of view. This study analyses the clinical, morphologic and cytogenetic features of the CDC observed and treated in our department. METHODS: From January 1995 to December 2002, among the 591 patients who underwent surgery for renal cancer, we observed 11 cases of CDC (prevalence 1.9%) treated either by radical (9 cases) or partial nephrectomy (2 cases). During radical nephrectomy a loco-regional lymphadenectomy was always performed. In the 9 cases observed after 1997, a complete cytogenetic analysis of the neoplastic karyotype was carried out. RESULTS: At pathological examination the disease was found to be confined to the renal capsule (TNM 1997 stage 1) in only 3 patients; venous neoplastic trombosis and nodal metastasis were present in 3 and 6 cases respectively; 2 patients showed distant metastases (lung, bone). Two of the patients affected with stage 1 tumours are still alive with no evidence of the disease at 48 and 88 months after surgery, while the third died following the systemic progression of a concomitant bladder carcinoma. One patient with stage 4 tumour (no. 11) is alive, but the follow up time is still limited (2 months). All the other 7 patients are dead after a mean survival time of 16.3 months (range 0-45). As for cytogenetic analysis, 2 CDCs didn't grow in culture and in one case no karyotype alterations were reported. In the remaining 6 cases hypodiploid stemlines and a homogeneous chromosome alteration pattern were observed, with multiple numerical and structural aberrations (mean 11.1, range 7-15) and the continuous involvement of chromosomes 1 and X or Y, both as traslocation and deletion/monosomy. Additional abnormalities of chromosomes 22 and 13 were found to be common but less frequent. CONCLUSIONS: The clinical behaviour of the CDC is aggressive and its prognosis is surely poor; surgical treatment seems to be curative only for organ-confined cancer, accounting for the minority of cases. This neoplasm is cytogenetically characterized by hypodiploid stemlines with common involvement of chromosome 1 and the autosomes.     

Collecting duct carcinoma presenting as a bleeding complicated renal cyst

Collecting duct carcinoma is a rare type of renal cell carcinoma. It is usually diagnosed pathologically and carries a poor prognosis. Renal cell carcinoma arising within a cyst is also rare. We report a case of collecting duct carcinoma presenting as a giant, bleeding complicated renal cyst with minimal solid component. The patient had a relatively long survival after nephrectomy.      

Genetic alterations and chemosensitivity profile in newly established human renal collecting duct carcinoma cell lines

OBJECTIVE

To determine the genetic alterations and chemosensitivity profile of collecting duct carcinoma (CDC) of the kidney, as it is a rare, highly aggressive malignant tumour with frequent distant metastases.

MATERIALS AND METHODS

We first established and characterized two human CDC cell lines designated AP3 and AP8, respectively. The CDC cell lines were assessed using microarray‐based comparative genomic hybridization and chemosensitivity testing.

RESULTS

The CDC cells grew in vitro as an adherent monolayer with epithelial morphology, but had different growth rates. The cell lines had the characteristic immunophenotype of CDC (high molecular weight cytokeratin‐+ve/cytokeratin 7‐+ve/vimentin‐+ve). Both cell lines shared copy number gains in chromosomes 20 and X. The loci showing a copy number gain were SOX22 at 20p tel, topoisomerse I (TOP1) at 20q12‐q13.1, TPD52L2 at 20q tel, 20QTEL14 at 20q tel, KAL at Xp22.3, STS 5′ at Xp22.3, OCRL1 at Xq25, AR3′at Xq11‐q12, and XIST at Xq13.2, respectively. Immunoblot analysis confirmed that the AP3 and AP8 cell lines showed moderate and high levels of TOP1 expression, respectively. By chemosensitivity testing, the AP8 cells were most sensitive to topoisomerase I and II inhibitors such as topotecan, epirubicin and doxorubicin, but the AP3 cells did not. The chemosensitivity to these drugs was paralleled by cell death via apoptosis.

CONCLUSION

The results suggest that TOP1 might be one of the molecular targets in AP8 CDC cells. Thus, these novel CDC cell lines will be useful for discovering therapeutic targets and developing effective anticancer drugs against CDC.     

Renal collecting duct carcinoma in an 8-year-old child

A fatal collecting duct carcinoma, presenting with pleural metastases, arose from the right kidney in an 8-year-old child. A distal nephron origin of the tumor is supported by positive tumor staining withUlex europaeus andArachis hypogaea, and a lack of staining withTetragonolobus lotus. The ultrastructural features of short stubby microvilli, smooth basal cell membranes, and lateral membrane infoldings also support a distal nephron origin (inner most inner medullary collecting duct). This rare childhood renal neoplasm behaved similarly to that reported in adults with metastatic disease at presentation and a short fatal clinical course.     

Impact of clinicopathological parameters in patients treated for renal cell carcinoma

PURPOSE: We determined the impact of clinical and pathological factors in the outcome of patients with renal cell carcinoma treated surgically. MATERIALS AND METHODS: We retrospectively reviewed the records of 230 consecutive patients after radical or partial nephrectomy. We analyzed clinical (incidental or symptomatic disease) and pathological (tumor size, histological type, Fuhrman nuclear grade, microvascular invasion and lymph node involvement) parameters. Disease-free and cancer specific survival curves were individualized for each parameter and on multivariate analysis. RESULTS: Median postoperative followup was 34.3 months, median time to recurrence was 22 months and mean overall survival was 130 months. A total of 40 patients (17.3%) presented with local and/or metastatic recurrence and 32 (13.9%) died of the disease. Five-year disease-free and cancer specific survival rates on univariate analysis were 56.7% and 64% for symptomatic tumors, 76.6% and 68% for clear cell carcinoma, 26.9% and 39% for sarcomatoid tumors, 34.7% and 47.5% for high grade tumors, 26.7% and 39.7% for microvascular invasion, 37.5% and 49.1% for tumors larger than 7 cm, and 11% and 32% for lymph node involvement, respectively. On univariate analysis patients with lymph node involvement and microvascular invasion had a poor prognosis. Multivariate analysis showed that the single independent prognostic factor was microvascular invasion. CONCLUSIONS: This study points out different clinical and pathological prognostic factors of survival in patients treated for renal cell carcinoma. Microvascular invasion was the only independent prognostic factor on multivariate analysis.     

Primary carcinoma of the cystic duct associated with sarcoid reactions: Report of a case

We report on an extremely unusual case of primary carcinoma of the cystic duct associated with sarcoid reactions. Although sarcoid reactions are known to occur occasionally with malignant tumors, primary carcinoma of the cystic duct is very rare, and to our knowledge, this is the first documentation of surgical treatment being performed for primary carcinoma of the cystic duct associated with sarcoid reactions. A 69-year-old woman with a mass in the right upper quadrant underwent cholecystectomy and hepaticocholedochal resection with dissection of the regional lymph nodes under the macroscopic operative diagnosis of primary carcinoma of the cystic duct. Microscopic examination of the resected lymph nodes disclosed sarcoid granulomas. According to our research of the literature, this is the 23rd surgically treated case of primary carcinoma of the cystic duct in Japan. The features of all 23 cases are reviewed following this case report.