Sarcomatoid features and lymph node-positive disease in chromophobe renal cell carcinoma

PurposeThe presence of sarcomatoid features and/or lymph node-positive disease may be associated with a worse prognosis in chromophobe renal cell carcinoma (ChRCC). We sought to better characterize patients' long-term outcomes with these features compared with those without these features.Materials and methodsWe identified 300 patients treated for sporadic, unilateral, nonmetastatic ChRCC between 1993 and 2019. Clinical and pathologic features were summarized, and cancer-specific survival (CSS) and recurrence-free survival (RFS) were analyzed using Kaplan-Meier plots. Cox regression analysis was performed to determine factors associated with recurrence. Patients with sarcomatoid features and/or nodal disease were grouped as high-risk in a secondary analysis.ResultsThe median age was 60 years, 43.7% were female, 29.3% had pT3/T4 disease, 3.3% had sarcomatoid features, and 4% had pathologic N1 disease. Sixteen patients were categorized as high-risk based on the presence of sarcomatoid features (n = 4), pathologic N1 disease (n = 6), or both (n = 6). There were 22 recurrences; the recurrence rate in the low-risk group was 4.9% and 50% in the high-risk group. 10-year RFS was 91.4% in the low-risk group and 34.4% in the high-risk group (P < 0.001). 10-year CSS was 96.4% in the low-risk group and 54.3% in the high-risk group (P < 0.001). In multivariable analysis, sarcomatoid features (HR 5.5, CI 1.5–20.2, P = 0.01) and pN1 disease (HR 16.5, CI 5.3–51.4, P < 0.0001) were independently associated with RFS.ConclusionsThe presence of sarcomatoid features and/or lymph node-positive disease portends a poor prognosis in ChRCC. Further studies evaluating the impact of novel therapeutic agents in these patients are warranted.     

Ultrastructural observations on mitochondria and microvesicles in renal oncocytoma, chromophobe renal cell carcinoma, and eosinophilic variant of conventional (clear cell) renal cell carcinoma

On light microscopic examination, the morphologically overlapping features of granular eosinophilic cytoplasm in renal oncocytoma and the eosinophilic variants of chromophobe renal cell carcinoma and conventional (clear cell) renal cell carcinoma may pose difficulties in diagnosis. We investigated the ultrastructure of 5 renal oncocytomas, 7 eosinophilic variants of chromophobe renal cell carcinoma, and 5 eosinophilic variants of conventional (clear cell) renal cell carcinoma. Special attention was paid to mitochondria and microvesicles and interrelations thereof. The electron microscopic features were correlated with the light microscopic findings. All of the tumors had abundant mitochondria. Although abundant microvesicles were present in all of the chromophobe renal cell carcinomas, scant numbers of microvesicles were also sometimes present in renal oncocytomas (2 of 5) and in the eosinophilic variant of conventional (clear cell) renal cell carcinoma (1 of 5). The mitochondria in all three types of renal neoplasms studied differed in morphology, being predominantly uniform and round with predominantly lamellar cristae in renal oncocytoma, variable in shape and size with predominantly tubulocystic cristae in chromophobe renal cell carcinoma, and swollen and pleomorphic with rarefied matrix and attenuated cristae in the eosinophilic variant of conventional (clear cell) renal cell carcinoma. Variable numbers of mitochondria in all of the chromophobe renal cell carcinomas had outpouchings of the outer membranes, some of which carried parts of inner membrane within them. These outpouchings closely resembled the nearby cytoplasmic microvesicles, as did the tubulocystic cristae of the mitochondria. Some microvesicles contained homogeneous, electron-dense, finely granular matrix, similar to that seen in mitochondria. In one of seven chromophobe renal cell carcinomas, microvesicles were present in rough endoplasmic reticulum, and in two others, mitochondria were present within some vesicles. These features strongly suggest a close relationship between the microvesicles and mitochondria. Based on the role of vesicle formation in normal mitochondriogenesis, and some of our observations, we propose that defective mitochondriogenesis may be the source of microvesicles in chromophobe renal cell carcinomas.     

囊性肾癌15例分析

目的 提高对囊性肾癌的认识。方法 对１９８２～１９９７年收治的１５２列囊性肾癌的临床特点、影像学、病理学特征及治疗预后情况进行回顾性分析。结果 术前影像学检查提示囊肿相关肾占生病变１２例,其中伴钙化５例,单纯性囊肿３例;术中活检发现癌变２例,多房囊肿性肾癌１例。１５例均行根治性肾切除术。１３例获随访,存活时间３个月～８年,平均３．２年。结论 囊性肾癌有其独特的临床,影像及病理学特征,对不符合典型单     

囊性肾癌的诊治(附12例报告)

目的　提高对囊性肾癌的认识。　方法　总结 12例囊性肾癌患者的诊断、治疗、预后等资料。术前诊断为复杂性囊肿 10例 ,9例术中行病理检查 ,8例报告为恶性。 12例中行根治性肾切除 7例 ,肾切除 3例 ,部分肾切除和囊肿去顶各 1例。　结果　 12例术后病理均为囊性肾透明细胞癌 ,平均随访 39.5个月 ,无肿瘤复发和转移。　结论　对可疑囊性肾癌者 ,术中须行病理检查 ;确诊者宜行根治性肾切除或部分肾切除。囊性肾癌预后较好     

Renal phenotypes related to hepatocyte nuclear factor-1beta (TCF2) mutations in a pediatric cohort

The hepatocyte nuclear factor-1beta encoded by the TCF2 gene plays a role for the specific regulation of gene expression in various tissues such as liver, kidney, intestine, and pancreatic islets and is involved in the embryonic development of these organs. TCF2 mutations are known to be responsible for the maturity-onset diabetes of the young type 5 associated with renal manifestations. Several observations have suggested that TCF2 mutations may be involved in restricted renal phenotypes. Eighty children (median age at diagnosis 0.2 yr) with renal cysts, hyperechogenicity, hypoplasia, or single kidneys were studied. Quantitative multiplex PCR amplification of short fluorescence fragments for the search of large genomic rearrangements and sequencing for the detection of point mutations were performed. TCF2 anomalies were detected in one third of patients (25 of 80). The main alteration was the complete deletion of the TCF2 gene detected in 16 patients. Family screening revealed de novo TCF2 anomalies in nine of 17 probands with a high prevalence of deletions (seven of nine). TCF2 anomalies were associated with bilateral renal anomalies (P < 0.001) and bilateral cortical cysts (P < 0.001). However, abnormal renal function, detected in 40% of patients, was independent of the TCF2 genotype. No difference in renal function or severity of renal morphologic lesions was observed between patients with a TCF2 deletion and those with point mutations. In conclusion, TCF2 molecular anomalies are involved in restricted renal phenotype in childhood without alteration of glucose metabolism. These findings have important implications in the diagnosis of patients with renal dysplasia with cysts and their follow-up.     

肾嫌色细胞癌的诊治（附4例报告）

目的 提高肾嫌色细胞癌的诊治水平。方法 回顾性分析4例肾嫌色细胞癌患者的临床资料。男3例，女1例，年龄34—76岁，平均52岁。结果 3例患者行肾癌根治术，1例行肾部分切除术。术后病理诊断为肾嫌色细胞癌，病理分期：pT1aNxMo 1例，pT1bNoMo3例。病理分级：G1 3例，G2 1例。免疫组织化学染色：CK8（低分子量细胞角蛋白）阳性，Vimentin（波型蛋白）阴性，Hale胶体铁阳性。随访4个月至8年，平均4年。4例患者均健在，无肿瘤复发或转移。结论 肾嫌色细胞癌是一种低度恶性的肾细胞癌，影像学检查对其诊断有重要帮助，确诊有赖于组织病理学检查，手术治疗后预后较好。     

A case report of chromophobe cell renal carcinoma arising from atrophic kidney during long-term haemodialysis

A 60-year-old woman with chromophobe cell renal carcinoma arising from the atophic right kidney during long-term haemodialysis was reported. The right renal tumour was detected incidentally by abdominal ultrasound examination. She received right nephrectomy through flank incision, and the pathological diagnosis was an eosinophilic variant of chromophobe cell renal carcinoma. Chromophobe cell renal carcinoma is a relatively rare subtype of renal cell carcinoma (5%), and the rate of this subtype on a long-term haemodialysis was quite low (0.6-0.7%), and almost all these patients had acquired cystic disease accompanied with haemodialysis. By contrast, our case occurred in the atrophic kidney (non-cystic kidney), and this might be the first case report of chromophobe cell renal carcinoma arising from an atrophic kidney in a patient on long-term haemodialysis.     

肾嫌色细胞癌影像学特点与外科治疗选择

目的 提高肾嫌色细胞癌的临床诊治水平. 方法 回顾性分析25例肾嫌色细胞癌患者临床资料.男13例、女12例,平均年龄51岁.左侧13例,右侧12例.无症状肾癌16例,有腰部不适、发热、肉眼血尿等症状9例.实验室检查发现肝功能异常1例、红细胞沉降率加快1例.结合文献复习讨论肾嫌色细胞癌的影像学特点与外科治疗选择. 结果 B超检查肿瘤主要表现为低回声、有包膜、血流信号不明显.CT及MRI扫描肿瘤特征为边界清楚、质地均匀(CT平扫为70%、MR为73%),出血、坏死和囊性变少见,增强扫描多为均匀强化(CT为65%、MR为67%)及轻度强化(CT为65%、MR为93%).肿瘤直径＞4.0 cm的22例患者行根治性肾切除术、≤4.0 cm且位于肾周边的3例行肾部分切除术.肿瘤平均直径7.6 cm,剖面质地多均匀、呈灰白色或暗红色,光镜下癌细胞呈板状或条索状排列、胞质苍白或呈嗜酸性,免疫组化染色Vimentin阴性、CK18阳性.TNM分期pT1a 8例、pT1b 9例、pT2 6例、pT3a 2例.23例获随访,平均随访28个月,无瘤生存22例,1例于术后58个月发现肺转移,经干扰素-α治疗、吉西他滨及氟尿嘧啶化疗3个月后死亡. 结论 肾嫌色纽胞癌患者多无症状、分期较早.MR和CT主要表现为边界清楚、质地均匀、强化不明显.手术方式选择应遵循肾癌的外科治疗原则.患者预后多良好,手术至出现转移的时间间隔较长,建议延长术后随访时间.     

Cystic renal cell carcinoma arising from multilocular cystic nephroma of the same kidney

Multilocular cystic nephroma is an uncommon benign entity grouped among the cystic non-genetic diseases. It is characterized by variable-sized, non-communicating cysts separated by irregular thin walled septa. Though multilocular cystic nephroma is usually considered a benign lesion, malignant changes in the cysts should not be overlooked.     

Acquired cystic disease of the kidney and renal cell carcinoma

This book is a well-presented and data-rich compendium on theproblem of acquired cysts and kidney cancers that occur in failedend-stage kidneys. Its Japanese author, Dr Isao Ishikawa, isthe world's authority on this subject. This book shows his comprehensive     

肾嫌色细胞癌的诊断和治疗（附五例报告）

目的 提高肾嫌色细胞癌的诊治水平。方法 回顾性分析5例肾嫌色细胞癌资料,结合文献复习进行讨论。结果 3例肾部分切除术,2例行根治性肾切除术,随访个月至4年,4例无瘤存活,1例于术后1年死于中风。结论 肾嫌色细胞癌是一种低度恶性的肾细胞癌,确诊有赖于影像学检查及典型病理表现,治疗以手术为主。     

肾嫌色细胞癌(附15例报告)

目的　提高肾嫌色细胞癌的诊治水平和对此类型肾癌的认识。　方法　回顾性分析15例肾嫌色细胞癌的临床资料。男 10例 ,女 5例。年龄 4 7～ 74岁 ,平均 5 7岁。均行根治性肾切除术。　结果　术后病理证实为肾嫌色细胞癌。病理分期 :pT1N0 M0 6例 ,pT2 N0 M0 5例 ,pT3bN0 M0 2例 ,pT1N2 M0 1例 ,pT2 N2 M0 1例。病理分级 :G2 10例 ,G3 5例。 11例获随访 ,随访 2～ 31个月 ,平均19个月 ,1例死于心脏病 ,1例局部复发 ,9例无瘤生存。　结论　肾嫌色细胞癌是一种具有特殊形态的少见肾癌类型。肾根治性切除术是治疗肾嫌色细胞癌的首选方法。与同期、同级的其他类型肾癌相比 ,肾嫌色细胞癌预后较好。     

Bilateral renal cell carcinoma and renal cell carcinoma in the solitary kidney

We report on 43 patients with renal cell carcinoma in a solitary kidney, 39 of whom underwent a potentially curative resection. Of 36 patients who had a nephron salvaging procedure only 4 required ex vivo surgical resection. The survival curves of patients with solitary or bilateral lesions are similar, and depend more on the adequacy of tumor resection and tumor stage than on the fate of the contralateral kidney. Crude survival in this series was 64 per cent for patients followed for more than 1 year.     

Cystic renal cell and squamous cell carcinoma of single kidney with concurrent transitional cell carcinoma of ipsilateral ureter and urinary bladder.

We report on a case of the simultaneous occurrence of cystic renal cell and squamous cell carcinoma in a single kidney in a patient with concurrent transitional cell carcinoma of the ipsilateral ureter and urinary bladder. A review of the literature reveals this to be the first such occurrence.