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.     

Synchronous ipsilateral conventional renal cell and transitional cell carcinoma

Simultaneous occurrence of renal cell carcinoma (RCC) and transitional cell carcinoma (TCC) in the same kidney is unusual. We report a 61-year-old man with ipsilateral synchronous renal adenocarcinoma and renal pelvic TCC. He was referred to our department for gross hematuria and right flank pain. CT and MRI studies revealed a 57 × 50 mm irregular and infiltrative upper right kidney mass with necrotic components. A right radical nephrectomy was done. Pathological diagnosis was a high grade tumor originating from just beneath the intact urothelium of renal pelvis and infiltrating through the parenchyma showing solid and occasional tubular growth patterns. A second tumor in close proximity to the first was reported as well differentiated RCC. This is a rare case of combined renal malignancies.     

Renal cell carcinoma with unusual metastasis to the gallbladder

Gallbladder involvement in patients with renal cell carcinoma (RCC) is extremely rare. We present a report of a 61-year-old man with a synchronous RCC metastasis to the gallbladder presenting as an intraluminal polypoid mass simulating primary gallbladder carcinoma. Enhanced abdominal computed tomography demonstrated a well-enhanced polypoid lesion in the gallbladder. Intraoperative rapid pathological examination of the gallbladder tumor showed clear cell-type cancerous cells. Microscopically, tumor cells of both the resected kidney and gallbladder had round uniform nuclei, clear cytoplasm, and well-defined cytoplasmic borders, forming alveolar patterns. Immunohistochemically, the tumor cells were negative for cytokeratin 7 (CK7) and carcinoembryonic antigen (CEA), which is usually positive in primary clear cell carcinoma of the gallbladder. Therefore, the final diagnosis was RCC with a synchronous gallbladder metastasis.     

Survival in patients with rare subtypes of renal cell carcinoma

OBJECTIVE: To evaluate the survival of patients with rare malignant histological subtypes of renal cancer. PATIENTS AND METHODS: The Heidelberg classification of renal cell carcinoma (RCC) divides tumours into clear cell carcinoma (CCC), papillary cancer (PC), chromophobic cancer (ChC) and collecting duct carcinoma (CDC). Sarcomatoid tumours are in a different subgroup treated as a final stage of histological progression. Between 1990 and 1997, 319 nephrectomies were undertaken because of RCC in 317 patients. In 42 patients (13%) the pathological findings showed other than CCC; in 13 PC was confirmed histologically, in nine ChC, in 11 a mixed type of CCC and sarcomatoid type, in seven a sarcomatoid tumour and in four, CDC. RESULTS: One patient of the 13 with PC and two of the nine with ChC died. The worst prognosis was in those with CDC, CCC-sarcomatoid and sarcomatoid tumours, as all these patients died. CONCLUSION: The histopathological differentiation of RCC into subtypes gives additional useful prognostic information.     

Active targeted therapy for metastatic collecting duct carcinoma of the kidney: a case report and review of the literature

Collecting duct carcinoma (CDC) is a rare and aggressive renal cell carcinoma (RCC) with extremely poor prognosis, which has been shown to have a poor response to several kinds of systemic therapy. Targeted agents have greatly changed the therapeutic landscape in advanced RCC. Nonetheless, patients with CDC are always excluded from the prospective trials with targeted therapies due to its rarity. We present a case of metastatic CDC that responded favorably to the multiple tyrosine kinase inhibitor, sorafenib, achieving a partial response in both lungs and retroperitoneal lymph nodes metastases. We also reviewed the limited number of reports of metastatic CDC treated with targeted agents and found that 33.33 % (4/12) of patients had favorable clinical activity. These suggest that targeted therapy should be considered for the treatment of metastatic CDC and its prospective evaluation is encouraged.     

Collecting duct carcinoma: a rare renal tumor

The most common primary malignant renal tumor is renal cell carcinoma (RCC), which accounts for 3% of all adult malignancies. Bellini duct carcinoma or collecting duct carcinoma is an unusual rare variant of RCC. This histologically distinct tumor is very rare, with less than 100 cases reported in the literature, and accounts for approximately 1% of all malignant renal epithelial tumors. We report two cases of collecting duct carcinoma and highlight the rarity of these tumors and their similarity to RCC.     

Association of EMMPRIN and fascin expression in renal cell carcinoma: correlation with clinicopathological parameters

EMMPRIN and fascin are important factors in tumor invasion and progression. We tested the hypothesis that expression of EMMPRIN and fascin correlate with clinicopathological parameters of renal cell carcinoma (RCC). Immunohistochemical analysis of EMMPRIN and fascin were performed in tissue microarrays of 100 surgical specimens, including 35 clear-cell RCC (CRCC), 21 clear-cell RCC with granular differentiation (GRCC), 12 chromophobe RCC (ChRCC), 8 papillary RCC (PRCC), 9 carcinoma of the collecting duct of Bellini (CDC), 10 clear-cell RCC with sarcomatoid differentiation (SRCC), and 6 metastatic RCC. Average immunoscores of EMMPRIN were 100.8 in CRCC, 195.2 in GRCC, 298.4 in ChRCC, 219.2 in PRCC, 186.1 in CDC, 226.9 in SRCC, and 151.7 in metastatic RCC. Among all included cases, average EMMPRIN immunoscores were 84.6 in grade I, 130.4 in grade II, 184.3 in grade III, and 223.5 in grade IV. Additionally, average immunostaining scores of fascin were 53.6 in CRCC, 289.3 in GRCC, 193.3 in ChRCC, 151.8 in PRCC, 181.3 in CDC, 275.4 in SRCC, and 131.7 in metastatic RCC. Average fascin immunoscores were 59.3 in grade I, 91.6 in grade II, 130.2 in grade III, and 194.7 in grade IV. Higher EMMPRIN and fascin immunoscores also correlated significantly with TNM stages and survival rates in RCC. Significant correlation was found between EMMPRIN and fascin expression. In conclusion, higher expression of EMMPRIN and fascin correlate significantly with histological grades, clinical stages, and survival rates of RCC     

嫌色细胞癌与透明细胞癌混合性肾癌的诊断

目的 探讨嫌色细胞癌与透明细胞癌混合性肾肿瘤的诊断及鉴别诊断。 方法 回顾性分析1例嫌色细胞癌与透明细胞癌混合性肾肿瘤的临床资料，结合文献复习进行讨论。结果 病理检查证实该肿瘤为嫌色细胞癌与透明细胞癌混合性肾肿瘤，免疫组织化学揭示嫌色细胞癌可能来源于肾集合小管，Hale胶体铁染色阳性。电镜下胞浆内见大量小囊泡是嫌色细胞癌特征表现。结论 该类肿瘤临床无特异性，需借助病理、免疫组织化学和超微结构特征等明确诊断及相关组织来源。     

Construction of diagnostic and subtyping models for renal cell carcinoma by genome-wide DNA methylation profiles

BackgroundRenal cell carcinoma (RCC) is one of the most common urological cancers and has a poor prognosis. RCC is classified into several subtypes, among which kidney renal clear cell carcinoma (KIRC) and kidney renal papillary cell carcinoma (KIRP) are the two most common subtypes. Due to the lack of adequate screening and comparative analysis of RCC subtypes, effective diagnosis and treatment strategies have not yet been achieved.MethodsIn this study, 450K methylation array data were collected from The Cancer Genome Atlas (TCGA). The ‘limma moderated t-test’ and LASSO were used to construct diagnostic and subtyping models, and survival analysis was conducted online by GEPIA.ResultsWe built a model with 15 methylation sites, which showed high diagnostic and subtyping performance in specificity and sensitivity. At the same time, for potential clinical usability, we calculated the diagnostic and subtyping scores to classify RCC from normal tissue and distinguish the different RCC subtypes. Additionally, the CpG sites were mapped to their corresponding genes, which could also be used to predict the prognosis of RCC.ConclusionsDifferent methylation sites can be used as diagnostic and subtyping markers that are specific to RCC and RCC subtypes (KIRC and KIRP) with high sensitivity and accuracy.     

Distribution of cytokeratins and vimentin in adult renal neoplasms and normal renal tissue: potential utility of a cytokeratin antibody panel in the differential diagnosis of renal tumors

Adult renal epithelial neoplasms (RENs) comprise several distinct clinicopathologic entities with potential prognostic and therapeutic differences. Individual cases can show overlapping morphologic features, necessitating the use of ancillary methods. The purpose of this study was to determine the diagnostic utility of cytokeratin (CK) subtype expression pattern in a wide range of adult RENs. RENs (including clear cell [conventional] renal cell carcinoma (RCC), papillary RCC, chromophobe RCC, renal oncocytoma, collecting duct carcinoma (CDC), renal medullary carcinoma (RMC), urothelial carcinoma, metanephric adenoma (MA), tubulocystic carcinoma (TC) (also known as low-grade collecting duct carcinoma), and mucinous tubular and spindle cell carcinoma) were immunostained for CK subtypes (CK5/CK6, 7, 8, 13, 14, 17, 18, 19, 20), high molecular weight CKs 1, 5, 10, 14 (HMWCK), and vimentin (Vim). The expression pattern of normal kidney was also examined and correlated with RENs. Although there is some overlap, subtypes of RENs show distinctive CK expression profiles that may be useful in several differential diagnostic settings. Clear cell RCCs typically showed a restricted expression pattern of CK8, CK18 and Vim. Papillary RCCs typically expressed CK7, CK8, CK18, CK19, and Vim and could be distinguished from MA (CK7-). Chromophobe RCCs were typically CK7+, CK8+, CK18+, and Vim-, and could be distinguished from oncocytomas (typically CK7-). In oncocytomas, nonspecific staining of unblocked endogenous biotin is a potentially significant diagnostic pitfall. CDC, RMC, and TC demonstrated similar CK expression profiles (with the exception of HMWCK expression limited to CDC), supporting a close relationship between these entities. A panel of CK5/CK6, CK17, and Vim may be helpful in distinguishing CDC (typically CK5/CK6-, CK17-, Vim+) and urothelial carcinoma (typically CK5/CK6+, CK17+, Vim-). In conclusion, CK expression patterns may be helpful in several differential diagnostic situations when dealing with adult RENs.     

Renal cell carcinoma with a huge solitary metastasis to the contralateral adrenal gland: A case report

Renal cell carcinoma (RCC) is capable of metastasizing to several organs. Synchronous isolated contralateral adrenal metastasis of the primary RCC is, however, very rare. Herein we report a case of RCC with a huge solitary metastasis to the contralateral adrenal gland that was surgically treated. We scheduled nephrectomy for the left primary RCC and adrenalectomy for the right adrenal tumor. However, at surgery we found a huge right adrenal tumor that had invaded the right kidney, right renal vein, and inferior vena cava. Therefore right nephrectomy was performed simultaneously with resection and reconstruction of the inferior vena cava. Pathological findings demonstrated that the left renal tumor and right adrenal tumor had the same histology. Although the patient required hemodialysis, he remains well at six months postoperatively. So far, there have been only two cases of a solitary contralateral metastatic adrenal tumor that was larger than the primary RCC, thus the present case is the third one.     

Collecting duct carcinoma associated with oncocytoma

Collecting duct carcinoma (CDC) is a rare, highly aggressive malignant neoplasm that arises from the collecting duct epithelium of the kidney. CDC was reported to coexist with renal cell and transitional cell carcinomas. We report a rare case of CDC associated with oncocytoma, confirmed by the characteristic histological appearance and immunohistochemistry. We also review the epidemiological, histological and immunohistochemical criteria for diagnosis, in addition to the genetic and cytogenetic aberrations reported in the literature. Identification and reporting CDC is important for the establishment of treatment strategies and monitoring prognosis.     

Renal cell carcinoma in a horseshoe kidney presenting as an acute,left sided varicocele

We present a rare case of renal cell carcinoma (RCC) in a horseshoe kidney presenting as an acute left sided varicocele. A left sided varicocele is a well-described presentation of RCC, usually caused by tumour thrombus extending along the renal vein with resultant testicular vein occlusion. However, in our case a tumour in the lower pole of a horseshoe kidney caused an acute varicocele by direct involvement and occlusion of the testicular vein.     

Rare presentation of renal cell carcinoma: Solitary sternal metastasis

Renal cell carcinoma (RCC) may present as metastatic disease. However, RCC with solitary sternal metastasis is rare. We report a rare case of RCC with synchronous solitary sternal metastasis. The patient underwent radical nephrectomy, sternal tumour resection and reconstruction as a one‐stage procedure. The role of open sternal biopsy is also described. Review of the literature was carried out and a reasonably lengthy survival was observed. We concluded that radical surgical resection and reconstruction may offer the best chance of survival in managing RCC with solitary sternal metastasis in renal cell carcinoma.     

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。     

Contralateral metachronous tumor occurrence is more frequently associated with distant metastases or postoperative intrarenal recurrence in renal cell carcinoma patients

Objective: To analyze the prognosis of patients with sporadic bilateral renal cell carcinoma (RCC). Methods: From January 1979 to December 2007, 984 patients with sporadic RCC underwent surgery at our department. Of these, 53 patients (5.7%) presenting with bilateral RCC were included in this retrospective analysis. Results: Thirty‐one of the 53 bilateral RCC patients had synchronous RCC, and 22 had metachronous RCC. Distant metastases by the time of the bilateral tumor occurrence were found in four patients (13%) in the synchronous group and in 10 patients (48%) in the metachronous group. No difference was found between the two groups in terms of overall survival. In contrast, survival after the second surgery in the metachronous group was significantly lower than that after the first surgery (P < 0.001) in the synchronous group (P = 0.02). In addition, the incidence of local recurrence after partial nephrectomy was higher in the metachronous group (26%) compared to the synchronous group (4%, P = 0.04) or the unilateral RCC patients (0.4%, P < 0.01). Conclusions: Metachronous occurrence of RCC in the contralateral kidney is associated with an unfavorable prognosis, suggesting that metachronous contralateral tumors might be metastasis of the original tumors. A stricter follow‐up schedule is advisable for metachronous bilateral RCC patients.     

Renal cell carcinoma of native kidney in renal transplant recipients

OBJECTIVE: To evaluate the prevalence, prognosis and possible risk factors of renal cell carcinoma (RCC) of the native kidney in renal transplant recipients. PATIENTS AND METHODS: We retrospectively re-examined the follow-up data of 373 consecutive renal transplant recipients at our institution between August 1993 and September 2004. We collected the data of all de novo RCC of the native kidney in the current analysis. RESULTS: Of the 373 patients examined, 12 tumours of the native kidney were diagnosed in 10 individuals. The mean ages at transplantation and diagnosis were 33 and 45.8 years, respectively. Thirteen malignancies were discovered fortuitously. Among the renal ultrasonograms there were two false-negative results. The mean tumour size was 21 mm. Nephrectomy was performed in all cases. Among the 12 kidney malignancies, there were five conventional RCCs and seven papillary RCCs. Half of all tumours were Furhman Grade 3 lesions, and pT1aN0M0 tumours also accounted for all malignancies in the current cohort. One of the 10 patients died, from progression of metastases 6 years after diagnosis. One patient had a local recurrence 2 years after diagnosis. The other eight patients were alive with no evidence of disease at the time of the current report. No significant relationship was detected between RCC occurrence and clinical patient characteristics. CONCLUSIONS: There appears to be a greater risk of RCC of the native kidney in patients with end-stage renal disease. The present results suggest that an annual examination of the native kidney before and after renal transplantation is essential.     

Renal cell carcinoma with solitary toe metastasis

Solitary metastases to the small bones and/or to the soft tissue of the hands and feet (acrometastases) are rare. We report a case of renal cell carcinoma (RCC) with big toe metastasis revealed before the primary tumor became apparent. The best treatment for a single metastasis is always surgical excision, regardless of the lesion being synchronous or metachronous. The biological behavior of metastatic RCC is unpredictable and only early diagnosis and treatment may favorably affect patient survival. Thus, metastatic RCC should be included in the differential diagnosis of all enlarging cutaneous nodules, wherever they develop.     

Renal cancer specific antitumor activities of a mouse monoclonal antibody K2.7 to human renal cell carcinoma]

We have prepared a mouse monoclonal antibody (mAb) K2.7 (IgG3) by immunizing mice with renal cancer cell (RCC) line OS-RC-2. In a serological analysis by protein A assay, 25 out of 31 RCC lines reacted with the mAb K2.7 but none of the 50 other cell lines from different organs except for 2 cell lines did. In immunohistological analysis by indirect immunoperoxidase assay, 66 out of 72 renal cancer tissues showed positive staining. Metastatic lesions of renal cancers also reacted similarly to the primary lesion. Some restricted normal tissues including tubules of normal kidney showed positive staining. Specific antitumor activities of mAb K2.7 against RCC lines were investigated in vitro by complement dependent cytotoxicity (CDC) and antibody dependent cell mediated cytotoxicity (ADCC) assays. In CDC assay, all of the 9 RCC lines were killed by mAb K2.7 and normal human serum, and killing activities of mAb K2.7 presumably depend on the number of antibody molecules bound to the cell surface. Sera from 9 patients with renal cancers including low and high stages showed the same killing activities to 3 RCC lines as normal human serum. In the ADCC assay, peripheral leukocytes (PBLs) from 4 healthy donors showed strong killing activities to RCC lines. Killing activity differed with the individual. PBLs from the same 9 patients as in the CDC assay showed significantly positive killing activity against 3 RCC lines. These findings suggest the usefulness of mAb K2.7 for the specific immunotherapy of renal cancer.