Journal of Kidney Cancer and VHL https://mail.jkcvhl.com/index.php/jkcvhl <p>Journal of Kidney Cancer and VHL (ISSN: 2203-5826) is indexed in <strong>PubMed</strong>, Emerging Sources Citation Index of <strong>Web of Science (ESCI)</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> <p>Journal of Kidney Cancer and VHL - <strong>2022 -Impact Factor - 1.6</strong></p> Codon Publications en-US Journal of Kidney Cancer and VHL 2203-5826 Recent Improvements in Adult Wilms Tumor Diagnosis and Management: Review of Literature https://mail.jkcvhl.com/index.php/jkcvhl/article/view/281 <p>Wilms tumor, also denoted as nephroblastoma, an embryonal type of renal cancer, is the most common cancer that affects children in the first 5 years of life. Wilms tumor is very rarely seen in adults. Both adults and children showcase varied clinical symptoms. The metastasis of tumor in both adults and children are not uncommon. Though histological differences between children and adults are insignificant, the prognosis of adult Wilms tumors compared to children is abysmal. Despite remarkable advancements in oncology, no standard treatment protocol exists for Wilms tumor in adults. Children Wilms tumor treatment protocol is currently followed for adults with some changes. In this article, we reviewed the available treatment options for Wilms tumor in adults and protocols followed widely.</p> Vishnupriya Sakthivel Adeeb Ismail Z Devashree Vijayabalan Copyright (c) 2023 Vishnupriya Sakthivel, Adeeb Ismail, Devashree Vijayabalan https://creativecommons.org/licenses/by-nc-nd/4.0 2023-08-11 2023-08-11 10 3 32 36 10.15586/jkcvhl.v10i3.281 Chromophobe Renal Cell Carcinoma with Sarcomatoid Differentiation https://mail.jkcvhl.com/index.php/jkcvhl/article/view/254 <p>Chromophobe renal cell carcinoma (chRCC) is one of the less common types of kidney cancer and generally portends a more favorable prognosis. RCC with sarcomatoid differentiation has a more aggressive clinical course with poor outcomes. Four cases of chRCC with varying degrees of sarcomatoid differentiation were retrospectively reviewed at our institution, and clinicopathologic data as well as clinical courses were reported. Patients with higher degrees of sarcomatoid differentiation and larger tumors at presentation generally had and worse overall survival. chRCC with sarcomatoid differentiation portends a poor prognosis with limited data on systemic treatment options for metastatic disease.</p> Benjamin J. Lichtbroun Brian Shinder Tina Gowda Sara Arnav Srivastava Biren Saraiya Tina M. Mayer Ryan Cristelli Evita Sadimin Robert E. Weiss Eric A. Singer Copyright (c) 2023 Benjamin Lichtbroun, Brian Shinder, Tina Gowda Sara, Arnav Srivastava, Biren Saraiya, Tina Mayer, Ryan Cristelli, Evita Sadimin, Weiss Robert, Eric Singer https://creativecommons.org/licenses/by-nc-nd/4.0 2023-07-08 2023-07-08 10 3 1 8 10.15586/jkcvhl.v10i3.254 Comparison of Posterior and Antero-Lateral Renal Tumors in Retroperitoneal Laparoscopic Partial Nephrectomy: A Propensity Score Matching Analysis https://mail.jkcvhl.com/index.php/jkcvhl/article/view/273 <p>This study aimed to compare the antero-lateral and posterior localized renal masses in laparoscopic partial nephrectomy with the retroperitoneal approach in terms of operative, functional, and oncological outcomes. Patients who underwent retroperitoneal laparoscopic partial nephrectomy by a single surgeon between January 2013 and January 2021 were included in the study. A one-to-one propensity score matching (PSM) analysis was conducted to obtain two balanced groups. The patients were divided into two groups as posterior and antero-lateral according to the localization of the mass. A total of 239 patients were included in the PSM analysis, with 65 patients allocated to each group. The mean operative time was 79.2 ± 11.2 min in the posterior group, while it was 90.0 ± 11.6 min in the antero-lateral group (P &lt; 0.001). Warm ischemia time was 15.9 ± 2.4 min in the posterior group and 18.6 ± 2.7 min in the antero-lateral group (P &lt; 0.001). The median decrease in eGFR at 1 year was 4.8 (IQR, 2.9–6.9) mL/min in the posterior group and 5.0 (IQR, 2.8–11) mL/min in the antero-lateral group (P = 0.219). The warm ischemia time and clamping technique were found to be significant factors for predicting eGFR change after surgery (β:0.693, 95% CI: 0.39–0.99, P &lt; 0.001; β:6.43, 95% CI: 1.1–11.7, P = 0.017, respectively). We report that retroperitoneal laparoscopic partial nephrectomy provided longer warm -ischemia and operative time for antero-lateral renal masses than posterior masses. However, long-term oncological and functional results were similar for both localizations.</p> Hakan Anıl Ali Yıldız Ahmet Güzel Serkan Akdemir Kaan Karamık Murat Arslan Copyright (c) 2023 Hakan Anıl, Ali Yıldız, Ahmet Guzel, Serkan Akdemir, Kaan Karamık, Murat Arslan https://creativecommons.org/licenses/by-nc-nd/4.0 2023-07-10 2023-07-10 10 3 9 16 10.15586/jkcvhl.v10i3.273 Hereditary Renal Cell Carcinoma: Is Age an Independent Criterion for Genetic Testing? A Large Cohort from a Latin America Referral Center https://mail.jkcvhl.com/index.php/jkcvhl/article/view/242 <p>Although age younger than 46 years has been an independent criterion for genetic testing in hereditary renal cell carcinoma (hRCC), there is a lack of evidence in the literature. This study aims to analyze whether a 46-year-old cut-off should be considered an independent genetic testing criterion and to elucidate risk factors predicting a positive genetic test. Observational study from January 2010 to December 2021. All patients under 46 years with a non-metastatic kidney mass and surgical indication were included. We assume patients who relapse in the first 5 years of follow-up could have a positive genetic test. As risk factors for relapse, ergo positive genetic test, we consider those patients who presented multifocal, bilateral, or previous renal tumor. Of 2,232 nephrectomies for kidney cancer, 301 patients met the inclusion criteria. The median follow-up was 60 months (IQR 29-101). The estimated five-year RFS was 94.4% (95% CI 91.3-97.5). Tumor size, previous renal tumor, multifocality, bilaterality, and pT3 or pT4 stage were independent recurrence risk factors. Genetic testing was performed on 24 patients. 10 patients had pathogenic variants in the test, 8 of which recurred during their life. 46-year-old cut-off has shown low performance in genetic testing. Therefore, we recommend that it be considered only if other hRCC risk criteria exist. Multifocality, bilaterality, and previous renal tumor could predict a positive genetic test.</p> Tomás Carminatti Patricio Aitor García Marchiñena Ignacio Pablo Tobia González Valeria de Miguel Marcelo Martín Serra Pablo Germán Kalfayan Alberto Manuel Jurado Copyright (c) 2023 Tomas Carminatti, Patricio Aitor Garcia Marchiñena, Ignacio Pablo Tobia, Valeria de Miguel, Marcelo Martin Serra, Pablo German Kalfayan, Alberto Manuel Jurado https://creativecommons.org/licenses/by-nc-nd/4.0 2023-08-01 2023-08-01 10 3 17 22 10.15586/jkcvhl.v10i3.242 Von Hippel–Lindau Disease (VHL): Characteristic Lesions with Classic Imaging Findings https://mail.jkcvhl.com/index.php/jkcvhl/article/view/293 <p>Von Hippel–Lindau disease (VHL) is a multisystem cancer syndrome caused by the inactivation of the VHL tumor suppressor gene and involves various organ systems including the central nervous system (CNS), endocrine system, and the kidneys. Tumors seen in patients with VHL disease can be benign or malignant and are usually multifocal, bilateral, and hypervascular in nature. As most lesions associated with VHL are asymptomatic initially, early diagnosis and the institution of an evidence-based surveillance protocol are of paramount importance. Screening, surveillance, and genetic counseling are key aspects in the management of patients diagnosed with VHL disease and often require a multidisciplinary approach and referral to specialized centers. This article will discuss the characteristic lesions seen with VHL disease, their diagnosis, screening protocols and management strategies, as well as an illustrative case to demonstrate the natural progression of the disease with classic imaging findings.</p> Suryansh Bajaj Darshan Gandhi Divya Nayar Ali Serhal Copyright (c) 2023 Suryansh Bajaj, Darshan Gandhi, Divya Nayar, Ali Serhal https://creativecommons.org/licenses/by-nc-nd/4.0 2023-08-03 2023-08-03 10 3 23 31 10.15586/jkcvhl.v10i3.293