Journal of Kidney Cancer and VHL https://jkcvhl.com/~jkcvhlco/index.php/jkcvhl <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> en-US sbryant@codonpublications.com (​Dr. Ulka Vaishampayan) sbryant@codonpublications.com (Scott Bryant, Managing Editor) Sat, 15 Aug 2020 15:17:19 +0000 OJS 3.2.0.3 http://blogs.law.harvard.edu/tech/rss 60 Renal Manifestations of Tuberous Sclerosis Complex https://jkcvhl.com/~jkcvhlco/index.php/jkcvhl/article/view/131 <p>Tuberous sclerosis complex (TSC) is a genetic condition caused by a mutation in either the <em>TSC1</em> or <em>TSC2</em> gene. Disruption of either of these genes leads to impaired production of hamartin or tuberin proteins, leading to the manifestation of skin lesions, tumors and seizures. TSC can manifests in multiple organ systems with the cutaneous and renal systems being the most commonly affected. These manifestations can secondarily lead to the development of hypertension, chronic kidney disease, and neurocognitive declines. The renal pathologies most commonly seen in TSC are angiomyolipoma, renal cysts and less commonly, oncocytomas. In this review, we highlight the current understanding on the renal manifestations of TSC along with current diagnosis and treatment guidelines.</p> Nikhil Nair, Ronith Chakraborty , Zubin Mahajan, Aditya Sharma , Sidarth K. Sehti, Rupesh Raina Copyright (c) 2020 Nikhil Nair, Ronith Chakraborty , Zubin Mahajan, Aditya Sharma , Siddarth K. Sehti, Rupesh Raina https://creativecommons.org/licenses/by-nc-nd/4.0 https://jkcvhl.com/~jkcvhlco/index.php/jkcvhl/article/view/131 Thu, 27 Aug 2020 00:00:00 +0000 Renal Cell Carcinoma with Direct Extension into the Gonadal Vein, Uterus, Fallopian Tube, and Bilateral Ovaries: A Case Report https://jkcvhl.com/~jkcvhlco/index.php/jkcvhl/article/view/130 <p>Renal cell carcinoma (RCC) with invasion into the renal vein is well described; however, invasion into the gonadal vein is a rare event with less than five cases reported in the literature. RCC occasionally presents with metastasis to the ovaries or the fallopian tubes, although this is also a rare occurrence. We present a case of locally advanced left RCC with direct extension into the ipsilateral gonadal vein with extension into the bilateral ovaries and uterus, which has not been previously described. Computed tomography (CT) in a 72-year-old female with a 35-pound weight loss indicated the presence of a 16-cm left renal mass with caudal tumor extension through the left gonadal vein and regional lymph-adenopathy. There was no evidence of distant metastasis, and she underwent an open left radical nephrectomy. Intraoperatively, she was found to have direct extension of the tumor through the left gonadal vein into the uterus, bilateral ovaries, and the left fallopian tube. All visible dis-ease was resected, and retroperitoneal and pelvic lymphadenectomy were performed. The patient had an uneventful hospital course. Pathology revealed clear cell RCC, Fuhrman grade 3. The final pathologic stage was pT4N1M1. The patient was ultimately noted to have pulmonary metastasis and was treated with immunotherapy with no evidence of disease progression.</p> Sarah Sweigert, Petar Bajic, Alessa Aragao, Maria Picken, Michael E. Woods Copyright (c) 2020 Sarah Sweigert, Petar Bajic, Alessa Aragao, Maria Picken, Michael E. Woods https://creativecommons.org/licenses/by-nc-nd/4.0 https://jkcvhl.com/~jkcvhlco/index.php/jkcvhl/article/view/130 Fri, 14 Aug 2020 00:00:00 +0000 Histologic Heterogeneity of Extirpated Renal Cell Carcinoma Specimens: Implications for Renal Mass Biopsy https://jkcvhl.com/~jkcvhlco/index.php/jkcvhl/article/view/134 <p>Pathologic characteristics of extirpated renal cell carcinoma (RCC) specimens &lt;7 cm were reviewed to get better information on technical nuances of renal mass biopsy (RMB). Specimens were stratified according to tumor stage, nuclear grade, size, histology, presence of lymphovas-cular invasion (LVI), necrosis, and sarcomatoid features. When considering pT1 (0–7 cm) tumors pT1b (4–7 cm), RCC masses were more likely to have necrosis (43% vs 16%, P &lt; 0.001), LVI (6% vs 2%, P = 0.024), high-grade nuclear elements (29% vs 17%, P &lt; 0.001), and sarcomatoid features (2% vs 0%, P = 0.006) compared with pT1a (0–4 cm) tumors. Additionally, pT3a tumors were more highly associated with necrosis (P = 0.005), LVI, sarcomatoid features, and high-grade disease (P for all &lt; 0.001) when compared to pT1 masses. For masses &lt;4 cm, pT3a cancers were more likely to demonstrate necrosis (38% vs 16%, P &lt; 0.001), LVI (10% vs 2%, P = 0.037), high-grade nuclear elements (31% vs 17%, P = 0.05), and sarcomatoid features (3% vs 0%, P = 0.065) compared to pT1a tumors. Similarly, for masses 4–7 cm, pathologic T3a tumors were significantly more likely to have sarcomatoid features (16% vs 2%, P &lt; 0.001) and LVI (28% vs 6%, P &lt; 0.001) compared to pT1b tumors. In summary, pT3a tumors and those RCC masses &gt;4 cm exhibit considerable histologic heterogeneity and may harbor elements that are not easily appreciated with limited renal sampling. Therefore, if RMB is considered for renal masses greater than 4 cm or those that abut sinus fat, a multi-quadrant biopsy approach is necessary to ensure adequate sampling and characterization of the mass.</p> Lauren M. Nahouraii, Jordan L. Allen, MD, Suzanne B. Merrill, MD, Erik B. Lehman, Matthew G. Kaag, Jay D. Raman, MD, FACS Copyright (c) 2020 Lauren M. Nahouraii, Jordan L. Allen, MD, Suzanne B. Merrill, MD, Erik B. Lehman, Matthew G. Kaag, Jay D. Raman, MD, FACS https://creativecommons.org/licenses/by-nc-nd/4.0 https://jkcvhl.com/~jkcvhlco/index.php/jkcvhl/article/view/134 Tue, 25 Aug 2020 00:00:00 +0000