Collecting duct renal cell carcinoma: a single centre series and review of the literature.

IF 1.4 Q3 UROLOGY & NEPHROLOGY Central European Journal of Urology Pub Date : 2022-01-01 Epub Date: 2022-09-06 DOI:10.5173/ceju.2022.0143
Wies Vanderbruggen, Marc Claessens, Vincent De Coninck, Aline Duchateau, Thomas Gevaert, Steven Joniau, Robert Hente
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引用次数: 2

Abstract

Introduction: Collecting duct, or Bellini duct, renal cell carcinoma (CDRCC) is a rare tumour, comprising only 0.4-2% of all renal cell carcinoma. The goal of this study was to evaluate the cases in our institution and look at current available literature.

Material and methods: We searched all data on renal cell tumours in our institution between 2011 and 2021 and identified four cases with confirmed CDRCC pathology. Important features were listed and analysed. We also reviewed current available literature and compared it to our case series.

Results: All cases were men with a median age of 63.5 years. All were symptomatic at presentation. Two patients presented with flank pain and two with gross haematuria. Three patients had stage IV disease at time of presentation and one stage III disease. All cases had clear Bellini duct renal cell carcinoma appearance on microscopy with infiltrative tubular architecture and high-grade nuclear features. Immunohistochemic (IHC) staining was performed for diagnostic confirmation. Three patients underwent radical nephrectomy and received adjuvant chemotherapy. One case had kidney biopsy for diagnostic confirmation and received first line chemotherapy. Immunotherapy or tyrosine kinase inhibitor (TKI) were started for second, third or fourth line of treatment. Median overall survival after diagnosis was 11 months.

Conclusions: CDRCC is a rare subtype of renal cell carcinoma with poor prognosis, typically presenting in a more advanced or metastatic stage. Diagnosis can be challenging. Multimodality treatment should be considered using radical surgery and systemic treatment.

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收集管肾细胞癌:单中心系列和文献回顾。
导言:收集管或贝利尼管肾细胞癌(CDRCC)是一种罕见的肿瘤,仅占所有肾细胞癌的0.4-2%。本研究的目的是评估我们机构的病例,并查看当前可用的文献。材料和方法:我们检索了我院2011年至2021年肾细胞肿瘤的所有数据,并确定了4例确诊的CDRCC病理。列举并分析了其重要特征。我们还回顾了当前可用的文献,并将其与我们的病例系列进行了比较。结果:所有病例均为男性,中位年龄63.5岁。所有患者在发病时均有症状。2例患者出现腹部疼痛,2例出现肉眼血尿。3例患者在发病时为IV期疾病,1例为III期疾病。所有病例镜检均有清晰的贝利尼管肾细胞癌表现,具有浸润性管状结构和高级别核征。免疫组织化学(IHC)染色进行诊断确认。3例患者行根治性肾切除术并辅助化疗。1例行肾活检确诊并行一线化疗。免疫疗法或酪氨酸激酶抑制剂(TKI)开始第二,第三或第四线治疗。诊断后的中位总生存期为11个月。结论:CDRCC是一种罕见的肾细胞癌亚型,预后较差,通常出现在较晚期或转移期。诊断可能具有挑战性。应考虑综合治疗,包括根治性手术和全身治疗。
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来源期刊
Central European Journal of Urology
Central European Journal of Urology UROLOGY & NEPHROLOGY-
CiteScore
2.30
自引率
8.30%
发文量
48
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