Journal of Kidney Cancer and VHL <p>Journal of Kidney Cancer and VHL (ISSN: 2203-5826) is indexed in <strong>PubMed</strong>,&nbsp;Emerging Sources Citation Index&nbsp;of <strong>Web of Science</strong>, and the Directory of Open Access Journals (<strong>DOAJ</strong>). Journal is dedicated for the dissemination of research findings in kidney cancer and VHL.</p> Codon Publications en-US Journal of Kidney Cancer and VHL 2203-5826 Different Treatments of Symptomatic Angiomyolipomas of the Kidney <p>Development of more sensitive imaging techniques has caused an increase in the number of diagnosed small renal tumors. Approximately 2–3% of these lesions are proved to be angiomyolipomas (AML), a rare benign tumor of the kidney sometimes causing pain and hematuria. The most required approach is observation, but in the case of recurrent symptoms or larger tumors, which may cause bleeding, a more active treatment is required. We present two cases of symptomatic AML tumors of different sizes in the kidney: one treated with transarterial embolization (TAE), and the other with percutaneous cryoablation (CRA). The lesions were diagnosed on the basis of contrast-enhanced computed tomography (CT) scan and magnetic resonance imaging (MRI). Both treatments proved to be effective and safe for treating renal AMLs. A follow-up carried out, based on contrast-enhanced CT scan, confirmed complete treatment of AML and decreased lesion size. There are myriad minimally invasive approaches for the treatment of renal AMLs, and the preservation of renal function remains a priority. The most popular treatment option is the selective renal artery embolization. Owing to its limited invasiveness, CRA could be an attractive option for the preventive treatment of AML.</p> Giuseppina Pacella Eliodoro Faiella Carlo Altomare Flavio Andresciani Gennaro Castiello Caterina Bernetti Bruno Beomonte Zobel Rosario Francesco Grasso Copyright (c) 2021 Giuseppina Pacella, Eliodoro Faiella, Carlo Altomare, Flavio Andresciani, Gennaro Castiello, Caterina Bernetti, Bruno Beomonte Zobel, Rosario Francesco Grasso 2021-10-19 2021-10-19 8 4 32 37 10.15586/jkcvhl.v8i4.181 Genetic Polymorphisms of the TGFB1 Signal Peptide and Promoter Region: Role in Wilms Tumor Susceptibility? <p>The aim of the present study was to investigate the rs1800468 (G-800A), rs1800469 (C-509T), rs1800470 (C29T), and rs1800471 (G74C) <em>TGFB1</em> genetic polymorphisms and their haplotype structures in patients with Wilms Tumor (WT) and neoplasia-free controls. The genomic DNA was extracted from 35 WT patients and 160 neoplasia-free children, and the <em>TGFB1</em> polymorphisms were genotyped by polymerase chain reaction, followed by restriction fragment length polymorphism. The haplotype structures were inferred, and permutation and logistic regression tests were performed to check for differences in haplotype distribution between the control and WT individuals. Positive associations were found in the recessive model for rs1800469 T allele (OR: 8.417; 95% CI: 3.177 to 22.297; P &lt; 0.001) and for the rs1800470 C allele (OR: 3.000; 95% CI: 1.296 to 6.944; P = 0.01). Haplotype analysis revealed a significant negative association between GCTG and WT (OR: 0.236, 95% CI: 0.105 to 0.534; P = 0.0002); by contrast, the GTTG haplotype was associated with increased risk for WT (OR: 12.0; 95% CI: 4.202 to 34.270; P &lt; 0.001). Furthermore, rs1800469 was negatively correlated with tumor size and a trend toward a positive correlation for capsular invasion was observed in the dominant model (Tau-b: −0.43, P = 0.02 and tau-b: 0.5, P = 0.06, respectively). This is the first study with rs1800468, rs1800469, rs1800470, and rs1800471 <em>TGFB1</em> polymorphisms in WT, and our results suggest that the <em>TGFB1</em> promoter and signal peptide region polymorphisms may be associated with WT susceptibility and clinical presentation.</p> Cintya Mayumi Ishibashi Carlos Eduardo Coral de Oliveira Roberta Losi Guembarovski Bruna Karina Banin Hirata Glauco Akelinghton Freire Vitiello Alda Losi Guembarovski Marla Karine Amarante Karen Brajão de Oliveira Marina Okuyama Kishima Carolina Batista Ariza Maria Angelica Ehara Watanabe Copyright (c) 2021 Cintya Mayumi Ishibashi, Carlos Eduardo Coral de Oliveira, Roberta Losi Guembarovski, Bruna Karina Banin-Hirata, Glauco Akelinghton Freire Vitiello, Alda Losi Guembarovski, Marla Amarante, Karen Brajão de Oliveira, Marina Okuyama Kishima, Carolina Batista Ariza, Maria Angelica Ehara Watanabe 2021-10-16 2021-10-16 8 4 22 31 10.15586/jkcvhl.v8i4.182 Predicting Strict Trifecta Outcomes after Robot-Assisted Partial Nephrectomy: Comparison of RENAL, PADUA, and C-Index Scores <p>Nephrometry scores are designed to characterize tumors and stratify the surgical complexity. It remains unclear as to which nephrometry score can accurately predict the surgical outcomes. We aimed to assess the utility of radius, exophytic/endophytic, nearness, anterior/posterior, location (RENAL), preoperative aspects and dimensions used for anatomic classifications (PADUA), and centrality index (C-index) nephrometry scores for predicting the strict Trifecta achievement from a single institution series robotic-assisted partial nephrectomy (RAPN). We retrospectively identified the prospectively maintained robotic surgery database records of 91 patients who underwent RAPN between June 2015 and September 2020 in Antalya Training and Research Hospital. The main outcome of the study was the achievement of strict Trifecta (negative surgical margin, no major urologic complications, warm ischemia time ≤25 min, and ≥85% preservation of estimated glomerular filtration rate). A multivariable analysis was performed to identify the factors of strict Trifecta success. The mean patient age was 55.82 ± 13.37 years with a median clinical tumor size of 3.5 cm (IQR 2.5–4.9). The median RENAL, PADUA, and C-index score were 7(IQR 6–8), 8(IQR 7–10), and 2.01(IQR 1.64–2.72), respectively. A strict Trifecta could be achieved in 54 patients (59.3%). Clinical tumor size (P = 0.011), RENAL risk groups (low:reference; intermediate; P = 0.040; high; P = 0.009), PADUA risk groups (low:reference; intermediate; P = 0.044; high; P = 0.001) and C-index risk groups (low:reference; high; P = 0.015) were the independent predictors of strict Trifecta attainment in the multivariate analysis. None of the nephrometry scores were a superior predictor compared to other nephrometry scores in comparative analysis. RENAL, PADUA, and C-index scores were all independent predictors of a strict Trifecta achievement. Our comprehensive comparison of the three scores identified that none of the nephrometry scores proved to be inferior to others nephrometry scores.</p> Kaan Karamık Yasin Aktaş Ahmet Gürkan Erdemir Ekrem İslamoğlu Mahmut Taha Ölçücü Çağatay Özsoy Murat Savaş Mutlu Ateş Copyright (c) 2021 Kaan Karamık, Yasin Aktaş, Ahmet Gürkan Erdemir, Ekrem İslamoğlu, Mahmut Taha Ölçücü, Çağatay Özsoy, Murat Savaş, Mutlu Ateş 2021-10-01 2021-10-01 8 4 1 12 10.15586/jkcvhl.v8i4.183 Evolving Patterns of Metastasis in Renal Cell Carcinoma: Do We Need to Perform Routine Bone Imaging? <p>Advance diagnostic and treatment modalities have improved outcomes for renal cell carcinoma (RCC) patients, but the prognosis for those with metastatic disease (mRCC) remains poor. As given metastatic distribution is critical in guiding treatment decisions for mRCC patients, we evaluated evolving metastatic patterns to assess if our current practice standards effectively address patient needs. A systematic literature review was performed to identify all publicly available prospective clinical trials in metastatic renal cell carcinoma (mRCC) from 1990 to 2018. A total of 16,899 mRCC patients from 127 qualified phase I–III clinical trials with metastatic site documentations were included for analysis for incidence of metastases to lung, liver, bone, and lymph nodes (LNs) over time. Studies were categorized into three treatment eras based on the timing of regulatory approval: Cytokine Era (1990-2004), vascular endothelial growth factor/tyrosine kinase inhibitor (TKI) Era (2005-2016), and immune checkpoint inhibitor/TKI Era (ICI-TKI, 2017-2018) and also classified as first-line only (FLO) or second-line and beyond (SLB). Overall, an increase in the incidence of bone and LNs metastases in FLO and SLB, and lung metastases in FLO, was seen over the three treatment eras. Generally, the burden of disease is higher in SLB when compared with FLO. Importantly, in the ICI-TKI era, the incidences of bone metastasis are 28% in FLO and 29% in SLB settings. The disease burden in patients with mRCC has increased steadily over the past three decades. Given the unexpectedly high rate of bone metastasis, routine dedicated bone imaging should be considered in all patients with mRCC.</p> Justin Lin Yue Zhang Wei Hou Qian Qin Matthew D. Galsky William K. Oh Che-Kai Tsao Copyright (c) 2021 Justin Lin, Yue Zhang, Wei Hou, Qian Qin, Matthew D. Galsky, William K. Oh, Che-Kai Tsao 2021-10-13 2021-10-13 8 4 13 19 10.15586/jkcvhl.v8i4.202 Routine Bone Imaging for Metastatic Renal Cell Carcinoma: Is it Time? <p>Current guidelines by the National Comprehensive Cancer Network recommend that, in addition to routine computed tomography (CT) imaging, bone imaging and brain magnetic resonance imaging (MRI) should be obtained when clinically indicated. In this issue of the <em>Journal of Kidney Cancer and VHL</em>, a systematic literature review of clinical trials of metastatic renal cell carcinoma (mRCC) patients evaluates the incidence of osseous, lymph node, and lung metastases (<a href="">1</a>). In particular, the analysis by Lin et al focuses on the changes in incidence over time. The study finds that the incidence of bone, lymph node, and lung metastases has increased over time. This increase is significant in osseous metastases specifically. These results lead to two provocative questions. First, why have osseous metastases increased in incidence over time? Second, does this finding warrant a more aggressive and uniform approach to imaging to identify osseous metastases sooner?</p> Mamta Parikh Copyright (c) 2021 Mamta Parikh 2021-10-14 2021-10-14 8 4 20 21 10.15586/jkcvhl.v8i4.207