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 Renal Cell Carcinoma Arising from Isthmus of Horseshoe K <p>The most common congenital renal fusion anomaly is the horseshoe kidney (HSK) occurring in about 1 in 600–700 individuals in the Indian population. HSKs are associated with problems such as renal stones, obstruction of uretero-pelvic junction causing stasis, and infection due to ectopic location of the kidneys, malrotation of the kidneys, and vascular changes. In general, normally developed kidneys have more incidents of renal cell carcinoma (RCC) as compared to HSKs. The major issue arises during surgery of HSK due to their altered anatomy and aberrant blood supply. We present a case of HSK with RCC located in the isthmus of a 43-year-old woman.</p> Sanjay M. Khaladkar Sai Sabari Vinay Kumar Parripati Deepak Koganti Satvik Dhirawani Urvashi Agarwal Copyright (c) 2023 Sanjay M. Khaladkar, Sai Sabari Vinay Kumar Parripati, Deepak Koganti, Satvik Dhirawani, Urvashi Agarwal 2023-05-02 2023-05-02 10 2 1 7 10.15586/jkcvhl.v10i2.267 Primary Well-Differentiated Neuroendocrine Tumor of the Kidney <p>Primary neuroendocrine tumors (NET) of the kidney are rare. They present with varied symptoms, making their diagnosis difficult clinically as well as pathologically. We present to you the case of a renal NET, which presented in a young female patient. A 48-year-old female patient came with an incidentally detected right renal mass during the evaluation of a nonspecific gynecological problem. She underwent contrast-enhanced computed tomography (CT) of the abdomen, which showed a 57*45*34 mm mass with enlarged retrocaval and aortocaval nodes (25*12 mm). Renal cell carcinoma was suspected as per the CT findings, and metastatic workup in the form of FDG PET CT was done in view of the unusually enlarged nodes. She underwent robot-assisted radical nephrectomy along with lymph node dissection. Surgery was uneventful, and she recovered well in the postoperative period. In the final pathology, there was confusion regarding the diagnosis, and further immunohistochemistry (IHC) was recommended by the pathologist. IHC showed synaptophysin positive, chromogranin negative, CD56 focally positive with Ki-67 of 2–3%, which was suggestive of low-grade NET of the kidney. Lymph nodes were negative. She was kept on follow-up and a Ga 68-DOTANOC scan at 3 months showed no evidence of disease. Diagnosis and management of NET of the kidney still remains a debatable and controversial topic in view of its rarity. High index of suspicion needs to be observed in patients presenting with carcinoid syndrome and a renal mass. Nuclear scans like PET scan and DOTANOC scan can accurately stage the disease. Management includes partial or radical nephrectomy depending on the tumor characteristics. Further studies are required to optimize the treatment protocols for these patients.</p> Vishnu Prasad Charakkulam Vijay Sreelakshmi K Ravi Chandran Shashank Agrawal Ginil Kumar Pooleri Amrita Sao Copyright (c) 2023 Vishnu Prasad , Charakkulam Vijay Sreelakshmi, Ravi Chandran, Shashank Agrawal, Ginil Kumar Pooleri, Amrita Sao 2023-05-11 2023-05-11 10 2 8 12 10.15586/jkcvhl.v10i2.277 Von Hippel-Lindau is Associated to Pancreatic Neuroendocrine Tumors: A Comprehensive Review <div id="abstract"> <p>Multiorgan tumors are a hallmark of the autosomal dominant genetic disorder known as Von Hippel-Lindau syndrome (VHL), which is typically the result of inherited aberrations of the VHL tumor suppressor gene. The most frequent cancer is retinoblastoma, which can also occur in the brain and spinal cord, renal clear cell carcinoma (RCCC), paraganglioma, and neuroendocrine tumors. There may also be lymphangiomas, epididymal cysts, and pancreatic cysts or pancreatic neuroendocrine tumors (pNETs). The most frequent causes of death are metastasis from RCCC and neurological complications from retinoblastoma or central nervous system (CNS). Pancreatic cysts are present in 35–70% of VHL patients. Simple cysts, serous cysts, or pNETs are possible presentations, and the likelihood of malignant degeneration or metastasis is no greater than 8%. Although VHL has been associated with pNETs, their pathological characteristics are unknown. Furthermore, it is unknown whether variations in the VHL gene cause the development of pNETs. Hence, this retrospective study was undertaken with the main aim to examine whether pNETs are connected to VHL from a surgical perspective.</p> </div> Danilo Coco Silvana Leanza Copyright (c) 2023 Danilo Coco, Silvana Leanza 2023-05-23 2023-05-23 10 2 13 20 10.15586/jkcvhl.v10i2.272