Journal of Kidney Cancer and VHL最新文献

{"title":"Do Primitive Neuroectodermal Tumors of the Kidney Have a Predilection for Inferior Vena Cava Involvement? A Case Series and Review of the Literature.","authors":"Sovan Hota,&nbsp;Sidhartha Kalra,&nbsp;Lalgudi Narayanan Dorairajan,&nbsp;Ramanitharan Manikandan,&nbsp;Sreerag Kodakkattil Sreenivasan","doi":"10.15586/jkcvhl.2020.153","DOIUrl":"https://doi.org/10.15586/jkcvhl.2020.153","url":null,"abstract":"<p><p>The primitive neuroectodermal tumor (PNET) of the kidney is an extremely rare neoplasm, the diagnosis of which mainly depends upon histopathology, immunohistochemistry (IHC), and cytogenetics. A handful of cases reported in the literature mention about aggressive features of this neoplasm. The purpose of our study was to review our experience in not only the diagnosis and management of the patients with renal PNET but also to highlight its propensity to involve inferior vena cava (IVC) and also present a rare occurrence of Ewing's sarcoma (ES)/PNET of the renal pelvis. The clinical, operative, and histopathology records of four patients of renal PNET treated between January 2017 and December 2019 were reviewed and data analyzed concerning the available literature. Out of the four patients treated, two had level III and IV IVC thrombus, and one had dense desmoplastic adhesions with the IVC wall. One of the cases had a rare presentation of ES/PNET of the renal pelvis. All patients were managed surgically, while only one patient received adjuvant chemotherapy and following up with remission for the last 2 years and 4 months. On IHC, cluster of differentiation-99 (CD-99) was positive in all patients, and three were positive for Friend leukemia integration-1. PNET of the kidney is primarily an immunohistopathological diagnosis. This neoplasm has an increased propensity for the local invasion of surrounding structures. A multimodality approach with surgery, chemotherapy, and radiotherapy could offer better outcomes, although the prognosis of these tumors remains poor.</p>","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"7 4","pages":"8-16"},"PeriodicalIF":1.6,"publicationDate":"2020-10-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7594836/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38600041","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Renal Manifestations of Tuberous Sclerosis Complex.","authors":"Nikhil Nair,&nbsp;Ronith Chakraborty,&nbsp;Zubin Mahajan,&nbsp;Aditya Sharma,&nbsp;Sidharth K Sethi,&nbsp;Rupesh Raina","doi":"10.15586/jkcvhl.2020.131","DOIUrl":"https://doi.org/10.15586/jkcvhl.2020.131","url":null,"abstract":"<p><p>Tuberous sclerosis complex (TSC) is a genetic condition caused by a mutation in either the <i>TSC1</i> or <i>TSC2</i> 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 manifest 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>","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"7 3","pages":"5-19"},"PeriodicalIF":1.6,"publicationDate":"2020-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7478169/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38400487","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Histologic Heterogeneity of Extirpated Renal Cell Carcinoma Specimens: Implications for Renal Mass Biopsy.","authors":"Lauren M Nahouraii,&nbsp;Jordan L Allen,&nbsp;Suzanne B Merrill,&nbsp;Erik Lehman,&nbsp;Matthew G Kaag,&nbsp;Jay D Raman","doi":"10.15586/jkcvhl.2020.134","DOIUrl":"https://doi.org/10.15586/jkcvhl.2020.134","url":null,"abstract":"<p><p>Pathologic characteristics of extirpated renal cell carcinoma (RCC) specimens <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 lymphovascular 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 < 0.001), LVI (6% vs 2%, P = 0.024), high-grade nuclear elements (29% vs 17%, P < 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 < 0.001) when compared to pT1 masses. For masses <4 cm, pT3a cancers were more likely to demonstrate necrosis (38% vs 16%, P < 0.001), LVI (22% vs 2%, P < 0.001), high-grade nuclear elements (45% vs 17%, P < 0.001), and sarcomatoid features (12% vs 0%, P < 0.001) compared to pT1a tumors. Similarly, for masses 4-7 cm, pathologic T3a tumors were significantly more likely to have sarcomatoid features (12% vs 2%, P = 0.006) and LVI (22% vs 6%, P = 0.003) compared to pT1b tumors. In summary, pT3a tumors and those RCC masses >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>","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"7 3","pages":"20-25"},"PeriodicalIF":1.6,"publicationDate":"2020-08-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7478168/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38400489","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Renal Cell Carcinoma with Direct Extension into the Gonadal Vein, Uterus, Fallopian Tube, and Bilateral Ovaries: A Case Report.","authors":"Sarah E Sweigert,&nbsp;Petar Bajic,&nbsp;Alessa Aragao,&nbsp;Maria Picken,&nbsp;Michael E Woods","doi":"10.15586/jkcvhl.2020.130","DOIUrl":"https://doi.org/10.15586/jkcvhl.2020.130","url":null,"abstract":"<p><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 lymphadenopathy. 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 disease 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>","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"7 3","pages":"1-4"},"PeriodicalIF":1.6,"publicationDate":"2020-08-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7478167/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38400486","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Surgical Outcome of Renal Cell Carcinoma with Tumor Thrombus Extension into Inferior Vena Cava and Right Atrium (Beating Heart Removal of Level 4 Thrombus): A Challenging Scenario.","authors":"Abdul Rouf Khawaja,&nbsp;Khalid Sofi,&nbsp;Yasir Dar,&nbsp;Muzaain Khateeb,&nbsp;Javeed Magray,&nbsp;Abdul Waheed,&nbsp;Sajad Malik,&nbsp;Arif Hamid Bhat,&nbsp;Mohd Saleem Wani,&nbsp;Akbar Bhat","doi":"10.15586/jkcvhl.2020.149","DOIUrl":"https://doi.org/10.15586/jkcvhl.2020.149","url":null,"abstract":"<p><strong>Aim: </strong>\"To evaluate oncological and surgical outcomes of different levels of tumor thrombus and tumor characteristics secondary to renal cell carcinoma (RCC)\".</p><p><strong>Materials and methods: </strong>Retrospective review from 2013 to 2020 of 34 patients who underwent radical nephrectomy with thrombectomy for RCC with tumor thrombus extending into the inferior vena cava (IVC) and right atrium (RA) at our center. Level I and most level II tumors were removed using straight forward occluding maneuvers with control of the contralateral renal vein. None of the patients had level III tumor extensions in our study group. For level IV thrombus, a beating heart surgery using a simplified cardiopulmonary bypass (CPB) technique was used for retrieval of thrombus from the right atrium.</p><p><strong>Results: </strong>\" Of the 34 patients with thrombus\", 19 patients had level I, 12 patients had level II, none had level III, and three patients had level IV thrombus. Two patients required simplified CPB. Another patient with level IV thrombus CPB, was not attempted in view of refractory hypotension intraoperatively. Pathological evaluation showed clear-cell carcinoma in 67.64%, papillary carcinoma in 17.64%, chromophobe in 5.8%, and squamous cell carcinoma in 8.8% of cases. Left side thrombectomy was difficult surgically, whereas right side thrombectomy did not have any survival advantage. Mean blood loss during the procedure was 325 mL, ranging from 200 to 1000 mL, and mean operative time was 185 min, ranging from 215 to 345 min. The immediate postoperative mortality was 2.9%. Level I thrombus had better survival compared to level II thrombus.</p><p><strong>Conclusion: </strong>Radical nephrectomy with tumor thrombectomy remains the mainstay of treatment in RCC with inferior venacaval extension. The surgical approach and outcome depends on primary tumor size, location, level of thrombus, local invasion of IVC, any hepato-renal dysfunction or any associated comorbidities. The higher the level of thrombus, the greater is the need for prior optimization and the adoption of a multidisciplinary approach for a successful surgical outcome.</p>","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"7 3","pages":"11-17"},"PeriodicalIF":1.6,"publicationDate":"2020-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7479807/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38400488","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A Case of Metastatic Hereditary Leiomyomatosis and Renal Cell Cancer Syndrome-Associated Renal Cell Carcinoma Treated with a Sequence of Axitinib and Nivolumab Following Cytoreductive Nephrectomy.","authors":"Ichiro Yonese,&nbsp;Masaya Ito,&nbsp;Kosuke Takemura,&nbsp;Takao Kamai,&nbsp;Fumitaka Koga","doi":"10.15586/jkcvhl.2020.148","DOIUrl":"https://doi.org/10.15586/jkcvhl.2020.148","url":null,"abstract":"Hereditary leiomyomatosis and renal cell cancer syndrome (HLRCC) associated renal cell carcinoma (RCC) is an aggressive form of type 2 papillary RCC caused by deficiency of the fumarate hydratase gene. For patients with metastatic disease, no standard treatment has been established with dismal prognosis. We report a case of metastatic HLRCC-associated RCC in a 65-year-old Japanese male whose clinical features mimicked advanced renal pelvic cancer. A durable response was achieved with a sequence of axitinib and nivolumab after cytoreductive and diagnostic nephrectomy. Their potential therapeutic roles in the management of metastatic HLRCC-associated RCC have been discussed based on its molecular and biological backgrounds.","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"7 2","pages":"6-10"},"PeriodicalIF":1.6,"publicationDate":"2020-07-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7479808/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38400485","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Management of Residual or Recurrent Disease Following Thermal Ablation of Renal Cortical Tumors.","authors":"Justin Loloi,&nbsp;W Bruce Shingleton,&nbsp;Stephen Y Nakada,&nbsp;Ronald J Zagoria,&nbsp;Jaime Landman,&nbsp;Benjamin R Lee,&nbsp;Surena F Matin,&nbsp;Kamran Ahrar,&nbsp;Raymond J Leveillee,&nbsp;Jeffrey A Cadeddu,&nbsp;Jay D Raman","doi":"10.15586/jkcvhl.2020.133","DOIUrl":"https://doi.org/10.15586/jkcvhl.2020.133","url":null,"abstract":"<p><p>Management of residual or recurrent disease following thermal ablation of renal cortical tumors includes surveillance, repeat ablation, or surgical extirpation. We present a multicenter experience with regard to the management of this clinical scenario. Prospectively maintained databases were reviewed to identify 1265 patients who underwent cryoablation (CA) or radiofrequency ablation (RFA) for enhancing renal masses. Disease persistence or recurrence was classified into one of the three categories: (i) residual disease in ablation zone; (ii) recurrence in the ipsilateral renal unit; and (iii) metastatic/extra-renal disease. Seventy seven patients (6.1%) had radiographic evidence of disease persistence or recurrence at a median interval of 13.7 months (range, 1-65 months) post-ablation. Distribution of disease included 47 patients with residual disease in ablation zone, 29 with ipsilateral renal unit recurrences (all in ablation zone), and one with metastatic disease. Fourteen patients (18%) elected for surveillance, and the remaining underwent salvage ablation (n = 50), partial nephrectomy (n = 5), or radical nephrectomy (n = 8). Salvage ablation was successful in 38/50 (76%) patients, with 12 failures managed by observation (3), tertiary ablation (6), and radical nephrectomy (3). At a median follow-up of 28 months, the actuarial cancer-specific survival and overall survival in this select cohort of patients was 94.8 and 89.6%, respectively.</p>","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"7 2","pages":"1-5"},"PeriodicalIF":1.6,"publicationDate":"2020-06-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7331942/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38152932","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Resistance to Pembrolizumab and Axitinib in Renal Cell Carcinoma: Clinical and Genomic Evaluation.","authors":"Panagiotis J Vlachostergios","doi":"10.15586/jkcvhl.2020.135","DOIUrl":"https://doi.org/10.15586/jkcvhl.2020.135","url":null,"abstract":"<p><p>Clear cell renal cell carcinoma (ccRCC) represents the most common subtype of renal cell carcinoma (RCC). In spite of recent advances in the treatment armamentarium and outcomes with the combined use of immune checkpoint and angiogenesis inhibitors, prediction of responses and selection of patients remain a challenge. This is a case of ccRCC with recurrence to the liver 1 year following right radical nephrectomy, who rapidly progressed on frontline therapy with axitinib/pembrolizumab. The clinical course and targeted tumor sequencing findings are discussed. In addition to established clinical prognostication in RCC, several surrogate markers of efficacy or/and resistance have been proposed for immunotherapy or/and anti-angiogenic therapy. Since the majority of patients will still progress after these combinations, it is becoming increasingly important to develop robust predictive biomarkers to guide patient selection and sequencing of targeted therapies.</p>","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"7 1","pages":"7-11"},"PeriodicalIF":1.6,"publicationDate":"2020-06-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7497900/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38526610","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The Year in Review for Renal Cancer.","authors":"Ulka Vaishampayan","doi":"10.15586/jkcvhl.2020.127","DOIUrl":"https://doi.org/10.15586/jkcvhl.2020.127","url":null,"abstract":"The treatment of kidney cancer has made some remarkable strides over the last few years. Two regimens received Food and Drug Administration (FDA) approval, multiple biomarkers were reported to show promise, and further enhancement and refinement of the prognostic characteristics occurred. The combinations of anti-angiogenic tyrosine kinase inhibitors with immune checkpoint inhibitors have rap-idly become the preferred therapies in the front-line setting of advanced renal cancer.","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"7 1","pages":"5-6"},"PeriodicalIF":1.6,"publicationDate":"2020-05-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/f5/44/JKCVHL-7-127.PMC7258384.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38019309","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Spontaneous Retroperitoneal Hemorrhage in a Patient with Acquired Cystic Kidney Disease.","authors":"Ahmed Kotb,&nbsp;Asmaa Ismail,&nbsp;Hazem Elmansy,&nbsp;Owen Prowse,&nbsp;Walid Shahrour","doi":"10.15586/jkcvhl.2020.123","DOIUrl":"https://doi.org/10.15586/jkcvhl.2020.123","url":null,"abstract":"<p><p>Spontaneous retroperitoneal hemorrhage (SRH) is a rare emergency. It is usually encountered in patients on hemodialysis and is associated with high rate of morbidity and mortality. This is a case from the emergency department. The patient had unstable vitals with SRH following dialysis. Immediate exploration and nephrectomy using transverse lateral lumbotomy incision were done. Patients on hemodialysis are at a risk of SRH and frequent surveillance is recommended. Acquired cystic kidney disease (ACKD) can develop in hemodialysis patients and put them at risk for bleeding. Transverse lateral lumbotomy may be a safe option for direct access to the kidney in emergency kidney surgery.</p>","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"7 1","pages":"1-4"},"PeriodicalIF":1.6,"publicationDate":"2020-04-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7243931/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38010050","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}