An unusual case of three synchronous tumors in a young woman

The occurrence in the same young patient of three synchronous tumors deriving from different embryogenic tissues and without a clear correlation with a common etiopathogenic factor is very unusual. We report a case of a Caucasian woman submitted to wide resection of a large retroperitoneal liposarcoma and right radical nephrectomy for suspected tumor infiltration. Histological examination of the right ureter and renal pelvis showed the presence of a multifocal urothelial carcinoma that was clinically asymptomatic. Two months later, during follow-up, chest X-ray and computed tomography documented a coin lesion of the upper left lung, confirmed by positron emission tomography. This nodule was surgically removed and examined histologically, resulting in a diagnosis of sclerosing hemangioma. The patient is alive without evidence of recurrent disease.     

Quelle place pour la radiothérapie et la chimiothérapie dans le traitement adjuvant des carcinomes urothéliaux des voies excrétrices urinaires supérieures ?

Urothelial carcinomas of the upper urinary tract are rare entities. Surgery remains the mainstay of the management. The use of others therapeutic modalities is not clearly defined yet. However, the frequency of local recurrence and locoregional encourage us to evaluate the indication of adjuvant therapies. We conducted a synthesis of key data in the literature on the use of chemotherapy and radiotherapy in the treatment of urothelial carcinoma of the renal pelvis and ureter. A literature search on PubMed was performed using the following keywords (MeSH) “urothelial carcinoma”, “upper urinary tract”, “radiation”, “chemotherapy”, and adjuvant.     

Therapeutic role of template-based lymphadenectomy in urothelial carcinoma of the upper urinary tract

Lymphadenectomy for urothelial carcinoma of the upper urinary tract has attracted the attention of physicians. The mapping study of lymphatic spread has shown that a relatively wide area should comprise the regional nodes for tumors of the right renal pelvis or the right upper two-thirds of the ureter. A prospective study showed that an anatomical template-based lymphadenectomy significantly improved patient survival in tumors of the renal pelvis. This benefit was more evident for patients with pT2 stage tumors or higher. The risk of regional node recurrence is significant reduced by template-based lymphadenectomy, which is likely to be associated with improved patient survival. The removal of lymph node micrometastases is assumed to be the reason for therapeutic benefit following lymphadenectomy. The number of resected lymph nodes can be used to assess the quality of lymphadenectomy, but not to determine the extent of lymphadenectomy. The guidelines currently recommend lymphadenectomy for patients with muscle-invasive disease, even though the current recommendation grades are still low. The present limitation of lymphadenectomy is the lack of standardization of the extent of lymphadenectomy and the randomized trials. Further studies are warranted to collect the evidence to support lymphadenectomy.     

Synchronous bilateral ureteral and renal pelvic carcinomas: incidence, etiology, treatment and outcome

BACKGROUND: Synchronous bilateral urothelial tumors of the upper urinary tract are very rare. The authors reported baseline and long-term follow-up data for all patients in western Sweden during a 28-year period. METHODS: The authors performed a clinical and histopathologic study of all patients in western Sweden who were diagnosed with a malignant neoplasm in the renal pelvis or ureter between 1971 and 1998. RESULTS: Of 936 patients, 15 (1.6%) had synchronous bilateral tumors. The incidence of such tumors decreased in each successive decade. Abuse of phenacetin-containing analgesics by patients also decreased during the study period, as did the incidence of renal papillary necrosis. The median age at diagnosis of bilateral tumors was 68 years, and 80% of the patients were male. Eleven patients had bilateral tumors of the renal pelvis, two had bilateral ureteral tumors, and two had tumors of the renal pelvis and contralateral ureter. Partial renal pelvic, ureteral, or kidney resection on at least one side was possible in eight patients, and four patients were left untreated on at least one side. Only three patients underwent bilateral nephroureterectomy. Twelve patients (80%) had bladder carcinoma diagnosed either before or after diagnosis of the upper tract tumors. The median survival period for the 11 patients who received surgery for their bilateral tumors was 84 months. CONCLUSIONS: The decreasing incidence of synchronous bilateral upper tract tumors may be related to the prohibition of phenacetin-containing analgesics in the 1960s. Partial resection with preservation of the renal parenchyma was possible in the majority of patients. Survival for patients with bilateral tumors did not differ from that of patients with unilateral tumors.     

Endoscopic diagnosis and treatment of upper-tract urothelial tumors. A preliminary report

The technique of transurethral ureteropyeloscopy allows many standard cystoscopic procedures to be extended into the upper urinary tract. This endoscopic method was used to evaluate 31 patients suspected to have urothelial malignancies of the ureter or renal pelvis. Twenty-eight of the patients had the procedure successfully completed (90%), 11 of whom were found to have urothelial tumors. Diagnostic ureteroscopic biopsy in three of these patients revealed high-grade, multifocal tumors and was followed by nephroureterectomy (two patients) or partial ureterectomy (one patient). However, in eight patients, ureteroscopy and biopsy revealed apparently localized, low-grade tumors which were treated by ureteroscopic fulguration or resection. The latter patients have undergone endoscopic surveillance every 3 months (average follow-up, 21 months). The technique of ureteropyeloscopy permits endoscopic access into the ureter and renal pelvis, enabling tissue diagnosis and better preoperative cancer staging without surgical exploration. Although follow-up is short, selected patients with low-grade tumors may be treated primarily by endoscopic means.     

腔内激光手术治疗肾盂输尿管肿瘤

目的:通过对无法行根治性手术的上尿路上皮肿瘤患者进行腔内红激光治疗情况进行总结,评价该术式的有效性与安全性.方法:对于我院2013年1月到2016年6月期间收治的肾盂、输尿管肿瘤,其中13例行腔内激光手术治疗,分别就术中情况、术后并发症及疗效进行总结.结果:13例患者中,肾盂肿瘤行经皮肾镜激光手术9例,输尿管肿瘤行输尿管镜激光手术4例,平均手术时间分别为(47.22±6.25)min、(25.0±4.84)min;平均出血量分别约为(133.33±24.94)ml、(40.0±7.07)ml;血尿及肾积水情况得到控制,5/13例呈现局部进展,1/13例术后10个月死亡;未出现严重并发症.结论:对于特殊情况下的肾盂、输尿管肿瘤患者,腔内激光手术具有创伤小,止血效果好,可以姑息性切除肿瘤,降低肿瘤负荷,保护肾脏功能,也能减缓肿瘤进展,可以作为这一类患者的首选治疗方案.     

Unusual clinical presentation of upper urothelial carcinoma in Taiwan

BACKGROUND: The unusual clinical picture of transitional cell carcinoma (TCC) in Taiwan appears to be different from that reported elsewhere. METHODS: The authors reviewed their experience of 135 cases of pathologically proven TCC of the upper urinary tract. Data regarding the gender ratio and clinical presentation were analyzed. RESULTS: The male to female ratio was found to be 1 to 1.3, showing a slight female predominance. The ratio of TCC of the renal pelvis to TCC of the ureter to TCC of the bladder was noted to be approximately 1.1:0.9:8.0. TCC of the renal pelvis accounted for 10.7% of all urothelial TCC, and for approximately 40% of all renal carcinomas. CONCLUSIONS: The results of the current study found that the gender incidence and ratio of renal pelvis TCC to all renal carcinomas are different in Taiwan compared with data presented in the majority of other studies.     

The pathology of urinary bladder lesions with an inverted growth pattern

Inverted lesions in the urinary bladder have been the source of some difficulty in urological pathology. The two common ones are von Brunn’s nests and cystitis cystic/cystitis glandularis, which are considered normal variants of urothelium. Apart from them, a number of other rare urothelial lesions with inverted growth pattern occur in the urinary bladder. Some of them are only reactive conditions, just as pseudocarcinomatous hyperplasia. Some are benign tumors, namely inverted papilloma. Whereas others are malignant neoplasms, including inverted papillary urothelial neoplasm of low malignant potential (PUNLMP), non-invasive inverted papillary urothelial carcinoma (low-grade and high-grade), and invasive urothelial carcinoma (inverted, nested and big nested variants). Because of the overlapping morphological features of all the inverted lesions mentioned above, even between high-grade invasive carcinoma and pseudocarcinomatous hyperplasia which are only a kind of reactive conditions, it is very important for the surgical pathologist to recognize and be familiar with these inverted lesions in urinary bladder. In this article, we review these spectrums of inverted lesions of the urinary bladder. Emphasis is placed on histogenesis, morphology, differential diagnosis of these lesions, and the pathologic grading of the non-invasive inverted neoplasms, such as inverted papilloma, inverted PUNLMP, non-invasive inverted papillary urothelial carcinoma with low-grade, and non-invasive inverted papillary urothelial carcinoma with high-grade.     

具有绒毛膜癌样特征的肾盂尿路上皮癌1例临床病理分析

目的:阐明具有绒毛膜癌样特征的肾盂尿路上皮癌临床病理特点。方法:回顾性分析1例具有绒毛膜癌样特征的肾盂尿路上皮癌患者的临床、影像、组织学和免疫组织化学特点。结果:患者CT检查发现右侧肾盂结节状类软组织密度影,遂行根治性肾脏切除术。术后病理检查显示肿瘤组织广泛坏死、出血,由类似于绒毛膜癌的细胞滋养层细胞和合体滋养层细胞组成,并与正常尿路上皮存在移行。免疫组织化学染色两类肿瘤细胞均阳性表达AE1/AE3、CK7及β-HCG,合体滋养层细胞阳性表达α-inhibin。经术后随访15个月,至今无瘤生存。结论:具有绒毛膜癌样特征的肾盂尿路上皮癌极为罕见,诊断时需要与转移性绒毛膜癌鉴别。     

Metastasis to the ovaries from transitional cell carcinoma of the bladder and renal pelvis: a report of two cases

Ovarian metastases from a primary urinary tract carcinoma are extremely rare. This can be difficult to distinguish from transitional cell carcinomas (TCC) of ovarian origin because of histologic similarity. A 65-year-old woman who was diagnosed with renal pelvis TCC 4 months prior was referred for evaluation of a left ovarian mass. A 47-year-old woman who underwent radical cystectomy due to bladder TCC 1 year ago was referred because of a right ovarian mass. Both patients underwent a bilateral salpingo-oophorectomy. The tumor cells had morphology identical to those of the primary urinary tract tumors. Gynecologic oncologists should consider metastatic TCC of the ovary from urinary tract origin, as well as breast, and gastrointestinal tract origins.     

经尿道输尿管口电切联合后腹腔镜治疗肾盂及上段输尿管尿路上皮癌

目的：评价经尿道输尿管口电切联合后腹腔镜治疗肾盂及上段输尿管癌的临床效果。方法：A 组15例肾盂及上段输尿管癌患者行经尿道输尿管口电切联合后腹腔镜的根治手术,B 组15例患者行传统开放手术,分析两组患者临床资料,将两组疗效进行比较。结果：两组患者均手术顺利。A 组手术时间、术后恢复时间、住院时间均短于 B 组,术中出血量少于 B 组,差异均有统计学意义（P <0.05或 P <0.01）。B 组出现切口感染2例,A 组未出现并发症,差异有统计学意义（P <0.01）。两组术后随访均未发现肿瘤转移,各有1例因未行规律膀胱灌注而出现膀胱内复发。结论：经尿道输尿管口电切联合后腹腔镜治疗肾盂及上段输尿管癌疗效安全、可靠,值得临床推广应用。     

Carcinomas of the Renal Pelvis,Ureters, and Urinary Bladder Share a Carcinogenic Field as Revealed in Epidemiological Analysis of Tumor Registry Data

BackgroundUrothelial carcinomas are the most common malignant tumors in the upper and lower urinary tract. Renal cell carcinomas (RCCs) have a different pathoepidemiologic incidence and characteristics. We describe a population-based approach of differentiating between urothelial and renal carcinomas as a basis to support shared morphologic phenotypes.Materials and MethodsData from 2000 through 2014 from the Surveillance, Epidemiology, and End Results Program of the National Cancer Institute were used to calculate the incidence rates for cancers of the kidney, renal pelvis, ureter, and urinary bladder. Graphic plots of the epidemiologic patterns were analyzed according to age frequency density and double logarithmic (log-log) plots of age-specific incidence rates and age of diagnosis.ResultsRCCs were initially more common than cancers of the urinary bladder, but after age 60, cancers of the bladder became more common with age-specific rates rapidly rising in all age groups. The age frequency density plot for RCC peaked earlier than for urothelial cancers indicating a different tumorigenic process. Log-log plots revealed near parallel proportional rate patterns for cancers of the renal pelvis, ureters, and urinary bladder, suggesting similar carcinogenic pathways among these tumors, whereas they were not parallel for RCCs. Similar slopes indicate that cancer incidence is increasing at similar rates regardless of the incidence of each cancer.ConclusionTumors that arise in the renal pelvis, ureters, and urinary bladder share a common carcinogenic field on the basis of pathoepidemiologic analysis. The definition of a carcinogenic field should expand to include epidemiological parameters as well as common morphologic and embryological patterns.     

A review of upper urinary tract cytology performance before and after the implementation of The Paris System

Urinary cytology (UC) is one of the primary diagnostic modalities used for the screening and surveillance of urothelial carcinoma. Despite its widespread use, UC has suffered from a lack of standardized or reproducible criteria and wide interobserver variability, particularly of the designation of atypical urothelial cells. The Paris System for Reporting Urinary Cytology (TPS), published in 2016, aimed to provide a standardized approach for evaluating UC by creating diagnostic categories with specific cytomorphologic criteria. Recent studies have primarily investigated the application of TPS on lower urinary tract specimens and have mostly shown that TPS implementation has improved the performance of UC specimens. Only a few studies have reported the impact of TPS on upper urinary tract (UUT) cytology. Additionally, there is uncertainty as to which cytological features are most predictive of high-grade urothelial carcinoma (HGUC) in the UUT. This review summarizes the literature regarding the utility and performance of UUT cytology and highlights findings before and after the implementation of TPS.     

Urine cytology in the detection of renal adenocarcinoma

Whereas urine cytology has proved to be of considerable diagnostic value for nonpapillary urothelial carcinoma, carcinoma in situ, and high-grade urothelial tumors, controversy has arisen over the accuracy of cytology in the diagnosis of renal neoplasms. To establish the reliability of urine cytology as a detection technique, 436 urine specimens from 59 patients with histologically proven renal adenocarcinoma were examined. Malignant cells in urinary sediment were found in 121 (27.75%) specimens from 31 (52.54%) patients. Seven of 20 (35%) patients with Stage I tumor showed neoplastic cells in 16 (16.5%) of 97 urinary samples. Positive cytologic features were found in 36.9% of 84 urinary specimens from 7 (50%) of 14 patients with tumors smaller than 5 cm. Using only imaging methods, the renal neoplasm was diagnosed in 58 (98.3%) cases. Urine cytology is, therefore, of little value in the diagnostic evaluation of known renal masses and in the detection of early-stage disease. In the current series there was not a single case with positive cytologic findings in which radiology did not reveal the tumor. The cytologic examination of the urinary sediment is unreliable in the diagnosis of radiologically unresolved cases of renal neoplasms. In 15 patients (44%) who had cancer cells in the urine the neoplasm had not invaded the renal pelvis. In 36% of patients with negative urine cytologic findings the renal pelvis was involved by renal adenocarcinoma. In the current study the desquamation of neoplastic cells in the urinary stream did not depend on tumor invasion of the renal pelvis. Based on the assumption that the primary objective of a screening procedure is to achieve an adequate sensitivity for early detection of disease, the results led the authors to conclude that the use of urine cytology as a possible screening test of renal adenocarcinoma is futile. Factors in the current study that diminished the practical value of cytologic examination of urinary sediment included the large number of specimens without malignant cells and the insensitivity of the procedure in diagnosing early renal adenocarcinoma of limited extent. Although this is the largest series reported, the number of cases considered was limited and further detailed studies are mandatory to definitively clarify the value of urine cytology in detecting renal adenocarcinoma.     

Evidence for oligoclonality and tumor spread by intraluminal seeding in multifocal urothelial carcinomas of the upper and lower urinary tract

Multifocality and recurrence of urothelial carcinoma may result from either the field effect of carcinogens leading to oligoclonal tumors or monoclonal tumor spread. Previous molecular studies, favoring the monoclonality hypothesis, are mostly limited to the urinary bladder. We investigated genetic alterations in a total of 94 synchronous or metachronous multifocal tumors from 19 patients with at least one tumor both in the upper and lower urinary tract. Loss of heterozygosity (LOH) was determined using eight markers on chromosome 9 and one marker on 17p13 (p53). Microsatellite instability was investigated at six loci and protein expression of MSH2 and MLH1 was evaluated by immunohistochemistry. In addition, exons 5-9 of the p53 gene were sequenced. Deletions at chromosome 9 were found in 73% of tumors and at 17p13 in 18% of tumors. There was no significant difference in the frequency of LOH in the upper and lower urinary tract. Deletions at 9p21 were significantly correlated with invasive tumor growth. The pattern of deletion revealed monoclonality of all tumors in nine patients. In five patients there were at least two tumor clones with different genetic alterations. In four of these patients the different clones occurred in the bladder and subsequently in the ureter and renal pelvis. All four patients with p53 mutations revealed identical mutations in all tumors. Thus, multifocal urothelial carcinomas are frequently monoclonal, whereas others show oligoclonality, providing molecular evidence for field cancerization. Intraluminal tumor cell seeding appears to be an important mechanism of multifocal occurrence and recurrence of urothelial carcinomas.     

关于上尿路移行上皮恶性肿瘤手术方式研究进展

上尿路移行上皮恶性肿瘤包括肾盂恶性肿瘤和输尿管恶性肿瘤.其标准术式为开放根治性肾输尿管切除术,随着腹腔镜技术不断发展,越来越多的泌尿外科医生将腹腔镜技术应用于上尿路移行上皮恶性肿瘤的治疗中,虽然腹腔镜手术较开放手术具有明显的优势,但操作技术难度较大,而机器人辅助腹腔镜技术的应用克服了许多复杂腹腔镜手术技巧的限制,从而为...     

A case of triple malignant tumors consisting of esophagus, stomach and malignant lymphoma with a histopathological feature of collision between gastric cancer and malignant lymphoma--a case report

We report a rare case of a collision between a gastric cancer and a malignant lymphoma with a wide systemic metastasis, combined with esophagus cancer, stomach cancer and malignant lymphoma. A 73-year-old man complained of gross hematuria and swelling of the right testis. Magnetic resonance imaging (MRI) revealed that both testes were swollen with unequal contrast and there were numerous tumors in the retroperitoneal space and pelvis. He was diagnosed with malignant diffuse large B cell lymphoma by immunostaining from the extirpated right testis. He received six cycles of R-CHOP therapy. After the second cycle, partial remission was recognized, but the tumors spread again by the fourth cycle. Thereafter, we performed MTX-HOPE therapy as a salvage therapy for four cycles. During this chemotherapy, he felt epigastralgia; esophagus cancer (squamous cell carcinoma) and stomach cancer (highly-differentiated adenocarcinoma) were found by upper endoscopy. However, the gastrointestinal cancer was inoperable, since the malignant lymphoma was progressive. His general status had been exacerbated, and he died about one year after he was diagnosed with malignant lymphoma. Pathological examination revealed that the adenocarcinoma had partly collided with the malignant lymphoma.     

Thorotrast-induced hepatic angiosarcoma, and combined hepatocellular and cholangiocarcinoma in a single patient

A 64-year-old man developed hepatic angiosarcoma, and combined hepatocellular and cholangiocarcinoma, 36 years after Thorotrast administration. The patient presented with a large mass in the right upper quadrant. His serum alpha-fetoprotein increased from 800 ng/ml to 51.2 micrograms/ml, and liver biopsy disclosed hepatocellular carcinoma. At autopsy, the liver had two different malignant neoplasms; angiosarcoma and combined hepatocellular and cholangiocarcinoma. Metastases of angiosarcoma to both lungs and of cholangiocarcinoma to periaortic lymph nodes were also seen. Interestingly, some neoplastic cells of angiosarcoma exhibited globular hyaline inclusions, which were Periodic-Acid-Schiff reaction positive and diastase-resistant.     

Rare coexistence of keratinizing squamous cell carcinoma with xanthogranulomatous pyelonephritis in the same kidney: report of two cases

Squamous cell carcinoma of urinary tract is a rarely encountered tumor. It is more frequently reported in urinary bladder and male urethra than renal pelvis. Squamous cell carcinoma of renal pelvis is usually associated with nephrolithiasis. However, coexistence of keratinizing squamous cell carcinoma with xanthogranulomatous pyelonephritis is exceedingly rare with only one case on record so far. We report two such cases detected incidentally in patients who have undergone nephrectomy for hydronephrosis. The post operative histological evaluation revealed unsuspected squamous cell carcinoma of renal pelvis with concomitant xanthogranulomatous pyelonephritis.     

A case of asynchronous development of transitional cell carcinoma in the bladder and renal pelvis after prolonged cyclophosphamide therapy

Urinary bladder cancers occurring after prolonged cyclophosphamide therapy are being increasingly reported. Cyclophosphamide-induced cancer in the upper urinary tract is not, however, generally recognized. We report a case of asynchronous development of transitional cell carcinoma in the bladder and renal pelvis, after prolonged cyclophosphamide therapy for non-Hodgkin's lymphoma. To date, at least 8 cyclophosphamide-related cancers have been reported in the upper tract. These cases are reviewed briefly.