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 Epidemiology and Clinicopathological Profile of Renal Cell Carcinoma <p>Renal cell carcinoma (RCC) accounts for 3% of all adult cancers and 85% of all kidney tumours. Incidence of RCC is lower in Asian region, particularly in India, probably due to lack of reporting. Most of the data about RCC are from Western countries; and data from India are scarce, especially regarding para-neoplastic syndromes. We sought to determine the epidemiology, clinicopathological profile and management of RCC in a tertiary care centre in Western India.<br />This was a retrospective study that involved data analysis of records of RCC patients who presented to our institution from April 2016 to Feb-ruary 2020. Laboratory investigations, including tests for paraneoplastic syndrome (PNS), and relevant radiologic investigations were performed and treatment was offered according to the stage, patient factors and available modalities.<br />A total 142 RCC patients were included in the study. The median age of presentation was 58 years. Most of the patients (67%) were symptom-atic, and 33% of the patients were asymptomatic, and the RCC was diagnosed incidentally. A large number of patients (56.3%) had PNS. The most common histopathologic type of RCC was clear cell carcinoma (68.8%), followed by papillary (20%) and chromophobe (8%) carcinoma. 40% of carcinomas with sarcomatoid differentiation were seen in patients under 50 years of age. Two cases of multicystic RCC were both seen in patients less than 50 years of age. 65.5% of the patients presented at Stage 1 and 2. Most surgeries (71.2%) were done in a minimally invasive manner.<br />A significant number of patients were asymptomatic, in which RCC was detected incidentally. The age of presentation was earlier, yet the patients had a higher tumour stage. More than half of the patients had PNSs. Despite growing trend towards Western data, the significantly higher number of patients with PNSs and early age of presentation suggest inherent differences in tumour biology, possibly related to differences in genetic and environmental factors.</p> Likhiteswer Pallagani Gautam Ram Choudhary Himanshu Pandey Vijay Kumar Sarma Madurri mahendra singh tanwar Prateek Gupta Nikita Shrivastava Gaurav Baid Meenakshi Rao Aasma Nalwa Puneet Pareek Sanjeev Misra Copyright (c) 2021 Likhiteswer Pallagani , Gautam Ram Choudhary, Himanshu Pandey, Vijay Kumar Sarma Madurri, Mahendra Singh, Prateek Gupta, Nikita Shrivastava, Gaurav Baid, Meenakshi Rao, Aasma Nalwa, Puneet Pareek, Sanjeev Mishra 2021-01-20 2021-01-20 8 1 1 6 10.15586/jkcvhl.v8i1.154 Bilateral Single-Stage Nephrectomy for Synchronous Bilateral Renal Cell Carcinoma <p>Bilateral synchronous renal cell carcinoma (RCC) is uncommonly encountered. Debate exists among urologists in managing these cases in a single surgery versus staged surgeries. We aim to report our experience in managing encountered cases using single-stage surgeries. Retrospective collection of cases with pathologically confirmed RCC that had single-stage bilateral renal surgery over the past 2 years. Three cases were identified. Patients were managed using bilateral transverse lateral lumbotomy. All patients did not have intraoperative or postoperative complications. Kidney function stayed stable after surgery. Single-stage bilateral renal surgery is a safe procedure. Bilateral transverse lateral lumbotomy allows for a fast and safe surgery with minimal complications. There is a possible histological disconcordance in bilateral synchronous RCC.</p> Ahmed Kotb Amer Alaref David Kisselgoff Asmaa Ismail Radu Rozenberg Nishigandha Burute Walid Shahrour Owen Prowse Hazem Elmansy Copyright (c) 2021 Ahmed Kotb, Amer Alaref, David Kisselgoff, Asmaa Ismail, Radu Rozenberg, Nishigandha Burute, Walid Shahrour, Owen Prowse, Hazem Elmansy 2021-01-25 2021-01-25 8 1 7 11 10.15586/jkcvhl.v8i1.151 Large Retroperitoneal Paraganglioma Associated with Germline Mutation of the Succinate Dehydrogenase Gene <p>Paragangliomas (PGLs) are rare neural tumors that can be benign or malignant and often associated with familial syndromes. We present a case of a 23-year-old male with a large retroperitoneal PGL found incidentally during the workup of elevated liver enzymes. After surgical excision, the patient was found to have an autosomal dominant mutation in the succinate dehydrogenase B (SDHB) gene, which when compared to sporadic PGLs or other familial syndromes is associated with a higher risk of tumor recurrence, occult metastasis, and development of other cancers. The patient’s first-degree relatives were recommended to undergo screening for the genetic mutation.</p> Wen Chen Philip Olson Rohith Arcot Huy Nguyen Faisal Qureshi Courtney Kokenakes Michael Cher Copyright (c) 2021 Wen Chen, Philip Olson, Rohith Arcot, Huy Nguyen, Faisal Qureshi, Courtney Kokenakes, Michael Cher 2021-01-25 2021-01-25 8 1 12 18 10.15586/jkcvhl.v8i1.129