clinical studies on primary renal pelvic tumors

The 15 cases of the primary renal pelvic tumors treated at our Hospital between 1974 and 1983, were reviewed retrospectively. The patients ranged in age from 41 to 74 years old (average: 58.3 years old). There were 11 males and 4 females, the ratio being 2.8:1.0. The affected side was left in 9 cases and right in 6 cases. The most frequent symptom was macrohematuria, which was seen in 12 cases (80%). The major finding of IVP was non-functioning kidney, which was seen in 8 cases (53.3%). Positive urinary cytology was obtained in 8 cases (53.3%). As the surgical method, total nephroureterectomy with bladder cuff was performed in 8 cases, nephroureterectomy in one case and nephrectomy in 6 cases. Histologically, 14 cases were transitional cell carcinoma and one case was squamous cell carcinoma. Simultaneous urothelial tumors were seen in the bladder of 2 patients. A subsequent ureteral tumor was found in one of the 7 cases in which ureters were resected incompletely, and subsequent bladder tumors were found in 8 of the 15 cases receiving surgical treatment in the follow-up period. All of tumors were found within 2 years after operation. Over-all actual survival rates at 1, 3 and 5 years were 87%, 67%, 48%, respectively. Three and 5 year actual survival rates were 100%, 100% respectively for the low stage group and 59%, 29% respectively for the high stage group. Three and 5 year actual survival rates were 100%, 78%, respectively for the low grade group and 44%, 27% respectively for the high grade group. Among several factors, stage and grade of the tumor were the most influencing factors for prognosis.     

泌尿男生殖系统淋巴瘤的临床特征及预后分析

目的探讨泌尿男生殖系统淋巴瘤患者的临床特征,并对其预后进行分析。方法回顾性分析2014年8月至2019年8月北京友谊医院收治的9例泌尿男生殖系统淋巴瘤患者的临床资料。男5例,女4例。平均年龄62(50~69)岁。肿瘤位于肾脏3例,膀胱2例,睾丸4例。首发症状:肾肿瘤3例中2例为发热(1例伴腹痛及体重减轻),1例为体检发现肾盂占位;膀胱肿瘤1例为肉眼血尿伴腹痛,1例为尿急、尿痛;4例睾丸肿瘤均为睾丸无痛性肿大。5例行CT检查见低密度肿物伴轻中度强化,4例睾丸肿瘤行B超检查提示不规则、不均质肿块内见血流信号。术前诊断均为泌尿男生殖系统肿瘤。6例接受手术,3例行病灶穿刺获取病理。9例中4例接受手术治疗联合化疗,2例仅接受化疗,2例仅接受手术治疗,1例未治疗。肾肿瘤3例中,1例行B超引导下病灶穿刺活检术,术后予环磷酰胺+阿霉素+长春新碱+泼尼松+利妥昔单抗(R-CHOP)方案静脉化疗;1例肾盂占位性病变者行腹腔镜肾输尿管全长切除术,术后予R-CHOP方案静脉化疗;1例行B超引导下病灶穿刺活检术,术后患者拒绝进一步治疗。2例膀胱肿瘤者均行TURBT,术后均拒绝放化疗。4例睾丸肿瘤者中,3例行患侧睾丸切除术,1例行B超引导下病灶穿刺活检术;3例行R-CHOP方案化疗,1例于外院行化疗及对侧睾丸放疗,具体方案不详;4例均行鞘内注射化疗药物(甲氨蝶呤15 mg+阿糖胞苷50 mg+地塞米松5 mg)预防中枢神经系统浸润。结果本组9例术后恢复顺利,无手术及穿刺相关并发症发生。术后病理诊断均为非霍奇金淋巴瘤,其中8例为弥漫性大B细胞淋巴瘤,1例睾丸淋巴瘤为间变性大细胞淋巴瘤。按照Ann Arbor分期标准进行临床分期,确诊时Ⅰ~ⅡE期4例,ⅢE~ⅣE期5例。国际预后指数(IPI)评分0~2分6例,≥3分3例。随访时间平均18(6~66)个月。7例存活,其中3例完成化疗者中,2例完全缓解(睾丸淋巴瘤ⅡE期IPI评分1分和ⅣE期IPI评分2分各1例),1例部分缓解(肾盂淋巴瘤ⅣE期IPI评分3分);3例拒绝化疗者未见肿瘤复发或进展(膀胱淋巴瘤2例和肾盂淋巴瘤1例,均为ⅠE期IPI评分1分);1例化疗2个周期后因出现细菌真菌混合感染性肺炎停止化疗(睾丸淋巴瘤ⅢE期IPI评分3分)。肾淋巴瘤ⅢE期IPI评分3分1例和睾丸淋巴瘤Ⅲ期IPI评分2分1例,分别于确诊后6个月、17个月因肿瘤进展死亡。结论泌尿男生殖系统淋巴瘤以弥漫大B细胞淋巴瘤为主,临床表现特异性差,诊断时应根据症状及影像学表现与其他常见肿瘤进行鉴别,避免误诊误治。组织病理学是诊断的金标准,R-CHOP方案化疗为首选治疗手段。     

A study of postoperative adjuvant chemotherapy of advanced urinary epithelial cancer

The effect of postoperative adjuvant chemotherapy was studied in 22 cases of advanced urinary epithelial cancer. Vincristine, mitomycin C and bleomycin (VMB) was administered in combination to 9 prophase cases from December, 1980 to March, 1982 and cis-dichlorodiamine platinum, peplomycin and mitomycin C (PPM) in combination to 13 anaphase cases from April, 1982 to November, 1984. The site was renal pelvic cancer in 3 cases, cancer of the ureter in 3 cases, cancer of the bladder in 13 cases, cancer of the pelvis, ureter, and bladder in 1 case, and recurrence of pelvic cancer following bladder cancer in 2 cases. The degree of invasion was pTa in 2 cases, pT1 in 1 case, pT2 in 1 case, pT3 in 11 cases and pT4 in 5 cases. Lymph node metastasis had occurred in 9 cases, no metastasis in 8 cases and it was unclear in the remaining 6 cases. The mean observation period was 16.5 months; 10 patients were alive without any tumors, one patient was alive with a tumor, 11 patients died of cancer, and one patient died intercurrently. The mean postoperative survival period in the mortality cases was 14.5 months. According to the classified type of chemotherapy received, there were 3 out of 9 cases (33.3%) who survived without tumors after receiving VMP and 7 out of 13 cases (53.8%) in the PPM group who survived without tumors. Although a simple comparison cannot be made, it appears that PPM therapy is superior. No severe side-effects were observed.     

膀胱鳞状细胞癌和膀胱腺癌的临床病理特征及预后分析

目的:探讨膀胱鳞状细胞癌(SqCC)和膀胱腺癌的临床病理特征及预后相关因素。方法:回顾性分析郑州大学第一附属医院2011年10月至2019年1月收治的107例膀胱非尿路上皮癌(NUBC)患者的病例资料。男78例,女29例,男女比例为2.69∶1;中位年龄62.0(51.0,72.0)岁。根据组织学类型将患者分为SqCC...     

A combined treatment with 8 MHz radiofrequency hyperthermia, HPC-adriamycin, immunotherapy, radiation, systemic chemotherapy and irradiation for invasive bladder carcinoma

Hyperthermia was induced for the treatment of invasive bladder carcinoma in order to study its usefulness. The subjects were 12 cases of invasive bladder cancer; including 5 cases of T2, 3 cases of T3, 2 cases of T4, and 2 cases of recurrence after total cystectomy. As previous treatment, 4 patients received radiotherapy and the other received TUR, systemic chemotherapy, and intravesical injection of anticancer drugs. For hyperthermia treatment, a Thermotron RF-8 was used for heating a deep seated tumor. Each case received hyperthermia 2 to 10 times. Combined therapy included injection of HPC-adriamycin into the urinary bladder in 5 cases, immunotherapy in 3 cases, M-VAC therapy in one case, radiotherapy in one case, radiotherapy and intra-arterial injection in one case, and Peplomycin and OK-432 local injection in one case. The treatment results showed a 75% effectiveness; with CR in 4 cases, PR in 5 cases, MR in 2 cases and PD in one case. Three patients died and 9 survived. Of four patients who had received radiotherapy as a previous treatment 3 cases obtained CR and one case MR. Therefore, it was considered that a favorable treatment effect with hyperthermia could be obtained after radiotherapy.     

泌尿系统原发性原始神经外胚层瘤临床病理分析

目的 探讨泌尿系统原发性原始神经外胚层瘤(primitive neuroectodermal tumor,PNET)的临床病理特征、免疫学表型、治疗方法及预后.方法 回顾性分析3例泌尿系统原发性PNET患者资料.3例均为男性,年龄分别为29、32和75岁.2例原发于肾脏,1例原发于膀胱.2例肾肿瘤大小分别为7.7 cm×6.2 cm和12.6 cm×9.4 cm,影像学检查提示肿瘤边界尚清,内部回声欠均匀.膀胱肿瘤大小为10.0 cm×10.0 cm,影像学检查提示膀胱壁不规则增厚,其内密度不均匀.2例肾肿瘤行肿瘤根治术,膀胱肿瘤行血块取出术及肿瘤活检术.结果 光镜下,瘤细胞为形态一致的小圆形或卵圆形,被纤维结缔组织分隔成实性片状或巢状,并形成假菊形团或Homer-Wright菊形团,核分裂象多见.免疫组化标记:3例肿瘤CD99、突触素和波形蛋白均为阳性.1例肾肿瘤Ki67阳性率＜5%,另1例80%阳性.3例病理诊断均为PNET.例1肾肿瘤患者未行化疗,于术后14个月复发死亡;例2肾肿瘤及例3膀胱肿瘤患者术后予以化疗,分别于术后4、6个月死亡.结论泌尿系统原发性PNET是一种少见的高度恶性软组织肿瘤,诊断主要依据病理形态学特征及免疫组化标记.目前治疗方法主要是手术加放、化疗.

Abstract：

Objective To explore the clinico-pathological features, immunophenotype, treatment and prognosis of urologic primary primitive neuroectodermal tumor (PNET). Methods The clinical data of 3 patients with urologic PNET were analyzed retrospectively. All patients were male, aged 29, 32 and 75 years respectively. Two of the lesions were located in the kidney, and the third was located in the bladder. The sizes of renal tumors were 7.7 cm×6.2 cm and 12.6 cm×9.4 cm respectively. Imaging examinations revealed a well-defined mass with inhomogeneous echo inside. The size of bladder tumor was 10.0 cm×10.0 cm. CT scan demonstrated irregular thickening of the bladder wall, and the density of the wall was inhomogeneous. In the 2 cases of renal PNET radical surgery was performed, while an emergency palliative surgery to remove a blood clot and biopsy were performed in the bladder PNET case. Results In light microscope, the tumors were characterized by uniform small round or oval cells and nest-like or dense sheet structures surrounded by sparse fibrovascular stroma. Homer-Wright rosettes or pseudorosettes were observed, as well as mitoses. Immunohistochemical study revealed that all cases showed positive staining for CD99, synaptophysin and vimentin. One of the renal tumor cells showed positive for CD56, and the other renal tumor and urocystic tumor cells were focally positive for chromogranin A. Additionally, in 1 of the cases of renal tumor there was a high positive rate of 80% for Ki67 staining while the other case showed less than 5%. All 3 cases were eventually diagnosed as PNET. The first renal tumor case was not treated with radiotherapy and chemotherapy postoperatively, and the patient died of recurrence 14 months after surgery. Both the second renal tumor case and the bladder tumor case underwent chemotherapy postoperatively, and they died 4 and 6 months after surgery respectively. Conclusions The urologic primary PNET is a very rare, highly malignant soft tissue tumor, and the diagnosis must be based on pathologic findings and immunohistochemical phenotypes. The multimodal treatment for urologic primary PNET consists of surgery, chemotherapy and radiotherapy.     

肾移植术后并发自体泌尿系统恶性肿瘤22例的临床分析

目的 分析肾移植受者并发自体泌尿系统恶性肿瘤的临床特征.方法 回顾性分析单中心1945例肾移植受者的临床资料,其中发生自体泌尿系统恶性肿瘤22例(发生率为1.13％),占所有恶性肿瘤的56.4 ％(22/39).22例中肾乳头状腺癌、肾乳头状细胞癌、肾血管肉瘤各1例;肾盂移行细胞癌1例,肾盂输尿管移行细胞癌6例,输尿管移行细胞癌7例,肾盂输尿管膀胱移行细胞癌1例;膀胱恶性肿瘤4例(包括膀胱移行细胞癌3例、膀胱交界恶性肿瘤1例).22例中,以肉眼血尿为主要症状者17例,2例反复出现镜下血尿,只有3例无明显临床症状.患者的发病年龄为(54.3±12.3)岁,诊断肿瘤的中位时间为移植术后53个月.10例采用环孢素A+硫唑嘌呤+泼尼松预防排斥反应,12例采用环孢素A+吗替麦考酚酯+泼尼松.所有患者均接受手术治疗,其中3例肾脏恶性肿瘤患者接受了根治性肾切除手术,15例肾孟、输尿管肿瘤患者接受患侧肾、输尿管切除并膀胱袖状切除,4例膀胱恶性肿瘤患者中,3例接受经尿道膀胱肿瘤电切术,1例行膀胱部分切除术.结果 随访2～97个月,死亡9例,死亡时间为肿瘤手术后6～97个月,死亡原因为骨转移1例,肺转移1例,脑转移2例,肝转移2例,全身广泛转移3例.随访截止时存活13例,存活时间最长者为单纯膀胱肿瘤患者,存活92个月,存活超过4年者4例,存活超过1年者5例.结论 自体泌尿系统恶性肿瘤是肾移植术后的一个重要并发症;无痛性肉眼血尿是最常见的症状;根治性手术切除是最主要的治 疗手段.     

泌尿系小细胞癌的诊断及治疗（附11例报告）

目的：探讨泌尿系小细胞癌的诊治方法、疗效及预后。方法：回顾性分析我院2003年1月～2012年12月收治的t1例泌尿系小细胞癌的病理、临床特征、诊断方法、治疗方法等资料,并对患者治疗效果、生存期等进行分析。结果：11例患者,男9例,女2例,平均年龄66．9（57～83）岁,肿瘤位于膀胱5例（1例为女性）,右肾3例（1例为女性）,左肾1例,前列腺2例。按TNM分期,膀胱肿瘤：T2bNnM01例,T2bN1M0 1例,2例均行根治性膀胱全切＋肠代膀胱术,分别于术后24个月及13个月死亡;T2bN2M1 1例,T3bN2M1 1例,此2例行根治性膀胱全切＋放疗＋化疗,分别于术后9个月、12个月死亡;T4N2M1 1例,行放疗＋化疗,3个月后死亡。肾脏肿瘤：T1aN0M01例,T1nN0M0 1例,T2bN1M0 1例,T3bN2M1 1例,均行根治性肾切除术＋放疗＋化疗,3例于术后6个月、13个月、24个月死亡,1例至今4年尚存活。前列腺肿瘤：T1cN0M1b 1例,行前列腺电切＋放疗＋化疗;T3bN1M1c 1例,行放疗＋化疗,随访时分别死于术后25个月及15个月。11例患者生存期最短3个月,目前最长48个月,平均17．5个月;1年生存率63．6％（7／11）,5年生存率目前为0。结论：泌尿系小细胞癌恶性度高,预后差,手术联合放化疗可能会延长患者生存期、改善其生活质量。     

Bilateral Wilms' tumors: changing concepts in management

Bilaterality is uncommon in Wilms' tumor, being present in 4% to 8% of the cases. We report the combined experience of two children's hospitals in one city over a 20-year period. We encountered nine cases of synchronous bilateral nephroblastoma (National Wilms' Tumor Study 3, stage V). Age at diagnosis ranged from 9 to 41 months (mean 23 months). There were five girls and four boys. Associated findings include nephroblastomatosis in three cases (33%), one of which also had a familial history; undescended testis in two cases; and minor anomalies in two other cases. Surgical treatment consisted of unilateral nephrectomy with contralateral partial nephrectomy or tumorectomy in six cases, nephrectomy with contralateral biopsy only in two cases, and the other patient had bilateral biopsies initially, followed at a later date by partial nephrectomy on one side. All patients received chemotherapy; actinomycin D (AMD) only was used in the oldest case, vincristine and AMD in five cases, to which was added cyclophosphamide in one case and adriamycin in two. Seven patients received radiation therapy. Seven out of the nine patients survived more than 2 years (77%); five are well, off chemotherapy, with no evidence of disease from 4 to 11 years after diagnosis. Two patients suffered from chronic renal failure and one died from complications after renal transplantation more than 19 years after diagnosis. The two patients who died from their disease presented with more advanced tumor. Therefore, the agressiveness of multimodal therapy can be tailored according to stage and histology, and effective chemotherapy allows maximal preservation of renal parenchyma in patients with stage I and II tumors.     

Primary Ewing's sarcoma/primitive neuroectodermal tumor of the kidney: a clinicopathologic and immunohistochemical analysis of 11 cases

Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) is an extraordinarily rare primary tumor in the kidney and can be mistaken for a variety of other round cell tumors, including blastema-predominant Wilms' tumor (WT). Approximately 90% of ES/PNET have a specific t(11;22), which results in a chimeric EWS-FLI-1 protein. Immunohistochemistry for the carboxy-terminus of FLI-1 is sensitive and highly specific for the diagnosis of ES/PNET. WT-1, the WT-associated tumor suppressor gene, is overexpressed in WT but not in ES/PNET. No study has examined FLI-1 or WT-1 expression in renal ES/PNET. The clinicopathologic features of 11 renal ES/PNET were studied along with immunohistochemistry for cytokeratin, desmin, CD99, FLI-1, and WT-1. WT were also immunostained for CD99 (5 cases), FLI-1 (10 cases), and WT-1 (9 cases). The patients (6 men, 5 women) ranged from 18 to 49 years of age (mean, 34 yr). The mean tumor size was 11.8 +/- 3.8 cm (mean +/- standard deviation). Presenting symptoms included abdominal/flank pain and/or hematuria. Grossly, all tumors showed necrosis and hemorrhage, and 4 had cystic change. Microscopically, all tumors showed vaguely lobular growth, primitive round cells, and variable rosette formation. Epithelial, myogenous, or cartilaginous differentiation was not seen. Immunohistochemical results on the renal ES/PNET were cytokeratin (2/8 focal), desmin (0/9), CD99 (8/8), FLI-1 (5/8), and WT-1 (0/8). In comparison, the WT only rarely expressed CD99 (1/5) and did not express FLI-1 (0/10), but were usually WT-1-positive (7/9). Follow-up on 8 cases (mean, 28 mo; range, 6-64 mo) showed 4 lung and pleural metastases, 1 bone metastasis, liver metastasis, 2 local recurrences, and 5 deaths from disease (median time to death, 16.8 mo). No case had distant metastatic disease at presentation. Adjuvant therapy included chemotherapy (8 cases), radiation (3 cases), and bone marrow transplantation (1 case). Our study affirms a unique proclivity of renal ES/PNET for young adults and that it is a highly aggressive neoplasm, with rapid death in many cases, usually after the development of treatment-resistant lung metastases. These tumors must be distinguished from blastema-predominant WT and other primitive renal tumors that require different therapy. FLI-1 and WT-1 immunohistochemistry may be valuable in this differential diagnosis, given the known immunophenotypic overlap between ES/PNET and blastema-predominant WT with regard to CD99, cytokeratin, and desmin. The accurate distinction between these two entities has clear prognostic and therapeutic implications.     

Treatment strategy for nodular renal blastema and nephroblastomatosis associated with Wilms' tumor.

Nodular renal blastema and nephroblastomatosis were present in 8 of 118 patients (6.8%) with Wilms' tumor. Five of these 8 patients (63%) had bilateral Wilms' tumors. Two had hemihypertrophy. Preoperative renal angiograms were accurate in detecting these metanephric anomalies. The surgical approach consisted of removal of the most diseased kidney and biopsy for diffuse tumors and wedge resections for localized tumors for the remaining kidney. Postoperatively, radiation was administered when tumor extended outside the kidney. Chemotherapy consisted of vincristine and dactinomycin for 18 mo and adriamycin for 6 mo. This method of management resulted in tumor-free survival of these 8 patients for 1--44 mo (median 24 mo). Nodular renal blastema and nephroblastomatosis may possibly develop into Wilms' tumor. All of these three conditions respond to surgery, chemotherapy, and radiation. When a Wilms' tumor is encountered, it is better to explore and possibly biopsy the opposite kidney. There is a place for second-look laparotomy in this spectrum of congenital anomalies.     

单次与多次膀胱灌注化疗对上尿路尿路上皮癌术后复发膀胱癌的影响

目的研究单次与多次膀胱灌注化疗对上尿路尿路上皮癌术后复发膀胱癌的影响。方法研究对象为2017年1月至2019年1月入院的46例上尿路尿路上皮癌术后患者,按照化疗方案划分为对照组(23例)与研究组(23例)两组,对照组选用单次膀胱灌注化疗,研究组选用多次膀胱灌注化疗,比较两组术后不良反应发生率、腹膜后肿瘤复发率及膀胱癌发生率。结果对照组术后出现1例膀胱刺激征,不良反应发生率为4.35%;研究组术后出现1例少量血尿,1例下尿路刺激征,不良反应发生率为8.70%,两组术后不良反应发生率差异无统计学意义(P>0.05)。对照组术后1例患者复发腹膜后肿瘤,发生率为4.35%;研究组术后无1例患者复发腹膜后肿瘤,两组术后腹膜后肿瘤复发率差异无统计学意义(P>0.05)。对照组术后发生9例膀胱癌,发生率为39.13%;研究组术后发生2例膀胱癌,发生率为8.70%,研究组术后膀胱癌发生率明显低于对照组(P<0.05)。结论多次膀胱灌注化疗可有效降低上尿路尿路上皮癌术后患者的膀胱癌发生率,对于预防膀胱癌具有积极作用,值得推广应用。     

Pseudocarcinomatous epithelial hyperplasia in the bladder unassociated with prior irradiation or chemotherapy

Pseudocarcinomatous epithelial hyperplasia in the bladder is a little known phenomenon, recognized to be associated with prior irradiation and/or chemotherapy. Whether this process can occur outside of this setting has not been studied. We identified 8 of these cases mimicking invasive urothelial carcinoma from our consultation files from 07/04 to 07/06 with no prior history of radiation or chemotherapy. The mean age at diagnosis was 65 years (range, 42 to 81 y), with 5 of the 8 males. Seven patients had a potential etiology for these changes that could either have resulted in localized ischemia or injury to the urothelium. These included case 1: atrial fibrillation, hypertension, congestive heart failure, gastrointestinal bleeding, and coronary artery vascular disease; case 2: coronary angioplasty, atrial fibrillation, hyperlipidemia, and amputation of arm for ischemia; case 3: hypertension, uncontrolled diabetes, hyperlipidemia, and atrial fibrillation; case 4: underlying arteriovenous malformation of the bladder; cases 5 to 6: history of indwelling Foley catheter; and case 7: history of radical prostatectomy for prostate cancer but no radiation. One patient had no potential contributing factors. All 8 patients presented with gross hematuria. At cystoscopy, 7 patients had polypoid lesions with 1 appearing nonpolypoid. Histologically, all cases showed epithelial proliferation of urothelium with cells having prominent eosinophilic cytoplasm. This process that mimicked invasive cancer within the lamina propria was marked in 3 cases (38%). Moderate nuclear pleomorphism was seen in 6 cases (75%). Only 1 case revealed mitotic figures. Ulceration was seen in 1 case. All cases showed some degree of hemorrhage with hemosiderin deposition identified in 3 cases (38%). Fibrin deposition was present in 1 case within the stroma, 3 cases in the vessels, and 4 cases in both. Five cases show stromal fibrosis. Edema and vascular congestion were common features (90% and 100%, respectively). Six out of 8 cases were accompanied by moderate to marked acute and chronic inflammation. The original diagnosis included nested variant urothelial carcinoma (1 case), atypical suspicious for invasive carcinoma (5 cases), hemangioma (1 case), and eosinophilic cystitis (1 case). Patients were followed for a mean of 16.5 months (range, 10 to 34 mo), and none developed bladder cancer. As a rare response to ischemia and chronic irritation, pseudocarcinomatous epithelial proliferations in the bladder may be confused with invasive urothelial carcinoma. Pathologists must be aware of the histologic changes mimicking cancer, and recognize that it can occur outside of the setting of prior irradiation or chemotherapy.     

肾移植受者发生的恶性肿瘤

目的 提高对肾移植后发生恶性肿瘤的诊治水平。方法 总结18例肾移植受者术后发生恶性肿瘤的临床资料，并进行随访。结果 18例患者在发现肿瘤时，13例移植肾功能良好，5例血肌酐升高；有9例患者为晚期肿瘤，已发生肿瘤浸润或远处转移，其中8例在3个月内死亡；9例患者接受手术、化疗或放疗，除1例非何杰金氏淋巴肉瘤和1例基底细胞癌分别于治疗后7个月、13个月死于肿瘤转移外，其余7例至今仍带肾存活。结论 （1）肾移植受者免疫功能低下，易发生恶性肿瘤；（2）早诊断、早治疗是有效的治疗方法，预后良好；（5）肾移植术后肿瘤患者应减少免疫抑制剂用量，实体瘤应尽早手术。     

A clinical study on 34 cases of urothelial cancer of upper urinary tract

Thirty-four cases of tumor of the renal pelvis or ureter or both have been treated in our department during the past decade. The primary tumor was in the renal pelvis in 11 cases, in the ureter in 21 cases and in the ureter and renal pelvis in 2 cases, a co-existent tumor in the bladder was found in 4 cases. Seventeen patients had a tumor on the right side and 17 on the left side. The most frequent symptom was gross hematuria (70.6%) and flank pain was the presenting symptom in 7 cases (20.6%). On the intravenous pyelography, a filling defect in the renal pelvis or ureter (41.2%) and nonvisualization (53.0%) were frequent findings. Twenty-nine cases had undergone total nephroureterectomy with resection of a bladder cuff, 3 had simple nephrectomy and 2 had open biopsy alone. Postoperative radiation therapy was done in 1 case, chemotherapy in 10 cases, and 6 cases of them were treated by CAP therapy (cis-dichlorodiamine platinum, doxorubicin and cyclophosphamide). Actual and relative 5-year survival rates were 53.8% and 63.5%, and no significant difference was found in survival rate between the patients with renal pelvic tumors and those with ureteral tumors.     

复杂上尿路癌的多镜联合治疗

目的探讨孤立肾、慢性肾功能不全时肾盂内或双输尿管内复杂上皮癌患者多镜联合治疗的可行性。方法3例均以间断无痛性全程肉眼血尿入院,通过尿脱落细胞学、IVU、CT、膀胱镜、输尿管镜等检查确诊为尿路上皮癌。例1为左侧重复肾、双输尿管畸形,重复肾上肾盂内、双侧3个输尿管内均为上尿路癌,采用经皮肾镜和输尿管镜联合行左侧重复肾上肾盂、双侧3条输尿管内肿物切除术;例2为肾功能不全、右肾萎缩,发生左侧肾盂、膀胱癌,行膀胱镜肿瘤电切及输尿管镜、经皮肾镜联合左肾盂癌局部切除术;例3为左侧肾盂癌根治术后3年,右孤立肾再发肾盂癌,经输尿管镜及经皮肾镜行右肾盂癌切除术。3例均成功保留肾脏。结果术后病理检查均为尿路上皮癌。早期经肾造瘘灌注化疗1~2次,之后常规膀胱化疗,分别随访13、14、15个月,B超及CT检查无肿瘤复发及死亡。例3孤立肾患者术后2天、2周时经肾造瘘化疗,术后1个月拔出输尿管支架管,于第2日夹闭肾造瘘管,出现发热,输尿管镜检查考虑输尿管狭窄明显,留置支架管,以后定期更换,1周后拔除肾造瘘管。结论对于孤独肾、对侧肾无功能、肾功能不全、年龄大、伴有禁忌证的内科疾患的复杂上尿路癌患者,采用输尿管镜、经皮肾镜联合治疗是可行和安全有效的,但需要术后严格的随访和检查。     

Therapy and outcome in 51 children with mesoblastic nephroma: a report of the National Wilms' Tumor Study

Mesoblastic nephroma is a distinctive pathologic renal tumor with unique clinical, therapeutic, and prognostic patterns. This report reviews 51 patients (2.8% of 1905 patients submitted to NWTS with renal tumors) with this diagnosis from the NWTS contrasting this disease entity with Wilms' tumor. There were 33 males and 18 females, predominantly term babies born after uncomplicated pregnancies. The mean age at tumor excision was 3.44 +/- 0.6 mo (one child 9 yr). A palpable mass was the predominant presentation in 48 patients and in addition hematuria (9), hypertension (2), vomiting (3), and jaundice (1) were noteworthy. Diagnostic studies included IVP (49 positive, 2 negative) and ultrasound (15 positive, 1 negative). Adequate operative excision was achieved in 43 of 51 patients while 8 children had local extension and 10 had tumor spillage. Operation alone (23), predominantly since 1978, surgery plus chemotherapy (24), prior to 1978, and surgery, chemotherapy, and radiation therapy (4), prior to 1976, were the modes of therapy. The follow-up ranges from 4 mo to 11.5 yr. Survival was excellent; 50 patients survive (98%), only 1 dying of sepsis. These data suggest that mesoblastic nephroma contrasts with Wilms' tumor in that it occurs in a younger age group, has a benign biologic behavior, and a more favorable outcome. Aggressive multimodal treatment though utilized in the early years of the study is not essential to achieve this outcome.     

Prophylaxis of recurrence of superficial bladder tumor by intravesical chemotherapy

During the past 10 years, we have experienced 110 bladder tumor cases. Among them, 70 patients were diagnosed superficial bladder tumor. Of these 70 cases, 30 were treated with intravesical adriamycin (ADR) and peplomycin (PEP), 13 with ADR only and one case with PEP and remaining 26 with TUR and hydrostatic pressure technique. We studied the efficacy of combination intravesical chemotherapy with ADR and PEP and other treatments in the prevention of recurrence in the superficial bladder tumor cases. The recurrence rate during 3 years of each group, was 25% in the group treated with ADR and PEP, 35% with ADR and 55% in remaining group. 3 years recurrence rate in the group treated with ADR and PEP was significantly low than that in the group tread with TUR and hydrostatic pressure technique alone (Wilcoxon test). Side effects was pollakisuria, pain after micturition and others. Anaphylactic shock appeared in one case. From these results we concluded that intravesical chemotherapy with combined agents is more effective than that with a single one or no treatment after TUR.     

泌尿系统肉瘤样癌和癌肉瘤八例报告并文献复习

目的 探讨泌尿系统肉瘤样癌和癌肉瘤的组织学特点、临床表现、治疗和预后.方法 泌尿系统肉瘤样癌和癌肉瘤8例.其中膀胱肉瘤样癌4例,男3例,女1例,年龄58、63、78、79岁,均因无痛性肉眼血尿就诊,肿瘤直径平均4.5(1.5～6.7)cm,为不规则、广基的浸润性肿物.行膀胱部分切除术2例,TURBt 1例,膀胱癌根治术1例.肾肉瘤样癌1例,男,64岁,因肉眼血尿就诊,伴同侧肾上腺和胰腺转移,行肾癌根治术和胰腺体尾切除术.转移性右肾上腺肉瘤样癌1例,男,47岁,原发灶为左肺肉瘤样癌,行左肺下叶和右肾上腺切除术.肾盂癌肉瘤1例,女,64岁,表现为无痛性肉眼血尿,行左肾、输尿管及部分膀胱切除术.膀胱癌肉瘤1例,男,77岁,无痛性肉眼血尿,肿瘤直径2.5～3.0 cm,行TURBt术.8例均经病理检查确诊.8例均获随访,随访时间36～96个月.结果 膀胱肉瘤样癌4例中:1例G_3、T_(2a)者行TURBt,术后行丝裂霉素膀胱灌注化疗,随访96个月无瘤生存;1例63岁女性先行TURBt,术后病理为肉瘤样癌、T_1伴有原位癌(T_m),1个月后行膀胱癌根治切除术.随访36个月无瘤生存;1例T3a者行膀胱部分切除术,术后辅以放疗,随访36个月无瘤生存;1例T4a者行姑息性膀胱部分切除术,术后病理切缘阳性,术后2个月死于肿瘤多脏器转移.肾肉瘤样癌患者术后2个月开始化疗(盐酸吉西他滨加卡铂),又出现肝、肺转移,术后5个月死亡.转移性右肾上腺肉瘤样癌患者术后在外院行3个疗程化疗(顺铂加依托泊苷),5个月后出现左肾上腺转移,术后7个月死亡.肾盂癌肉瘤患者的肿瘤以软骨肉瘤为主,伴少许鳞状细胞癌及未分化癌,随访60个月无瘤生存.膀胱癌肉瘤患者肿瘤病理分期为T2a,主要是平滑肌肉瘤,伴有鳞状细胞痛和腺癌,术后予以全身化疗(盐酸吉西他滨加卡铂),随访12个月死于慢性阻塞性肺气肿、肺部感染.结论 泌尿系统肉瘤样癌和癌肉瘤少见,多见于老年男性,恶性程度高,浸润性强,预后差.肿瘤的病理分期和治疗是影响预后的因素,早期诊断和积极的综合治疗可望获得较好的治疗结果、延长患者生存期.     

Clear cell sarcoma of kidney in an adolescent and in young adults: a report of four cases with ultrastructural, immunohistochemical, and DNA flow cytometric analysis

Clear cell sarcoma of the kidney is a distinct, highly malignant pediatric neoplasm. Its occurrence in adults is extremely rare and the subject of isolated case reports. We present a series of four cases (three males and one female) identified in an adolescent and in young adults (16, 18, 20, and 25 years) with flank mass (three cases), hematuria (two cases), flank pain (two cases), and hypertension (one case). Three patients had stage III disease and one had stage I disease (National Wilms' Tumor Study staging system). All tumors had predominantly or exclusively the classic histology of a monotonous proliferation of uniform small round cells with evenly distributed fine chromatin, although focal microcyst formation (two cases) and spindled architecture (one case) (variant patterns) were also noted. Therapy in all cases consisted of surgery and chemotherapy with or without radiation. Follow-up data (29-202 months) showed distant metastases in all four cases, including the lung (four cases), bone (two cases), and the liver (two cases). Three patients died of disease at 29, 59, and 63 months (mean, 50.3 months), and one patient is alive with no evidence of disease at 202 months. Ultrastructural features included scattered primitive junctions, short and irregular cytoplasmic extensions, and scant to a moderate amount of mitochondria. Immunohistochemical study (three cases) showed immunoreactivity with vimentin (two cases) and no reaction with cytokeratin, epithelial membrane antigen, S-100 protein, or desmin. Flow cytometric analysis showed diploid DNA content in three primary tumors and tetraploidy in one metastatic tumor. The proliferative activity (S-phase fraction) was low to intermediate (mean, 9.8%). Our data suggest that clear cell sarcoma of the kidney in the young adult age group resembles its pediatric counterpart in ultrastructural and immunohistochemical characteristics, proclivity for skeletal and visceral metastasis, DNA diploid status with relatively low S-phase, and aggressive clinical course. Clear cell sarcoma of the kidney in adult patients, although rare, must be differentiated from sarcomatoid carcinoma, sarcomas, and round cell tumors because of its unique characteristics in comparison to other renal neoplasms.