Journal of Kidney Cancer and VHL最新文献

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Papillary Renal Cell Carcinoma in Lynch/Muir-Torre Syndrome with Germline Pathogenic Variant in MSH6 and Molecular Analysis: Report of a Case and Review of the Literature. Lynch/Muir-Torre综合征乳头状肾细胞癌伴种系致病变异MSH6及分子分析:1例报告及文献复习
IF 1.6
Journal of Kidney Cancer and VHL Pub Date : 2021-04-21 eCollection Date: 2021-01-01 DOI: 10.15586/jkcvhl.v8i2.175
Yu Yang, Shweta Dhar, Jennifer Taylor, Bhuvaneswari Krishnan
{"title":"Papillary Renal Cell Carcinoma in Lynch/Muir-Torre Syndrome with Germline Pathogenic Variant in <i>MSH6</i> and Molecular Analysis: Report of a Case and Review of the Literature.","authors":"Yu Yang,&nbsp;Shweta Dhar,&nbsp;Jennifer Taylor,&nbsp;Bhuvaneswari Krishnan","doi":"10.15586/jkcvhl.v8i2.175","DOIUrl":"https://doi.org/10.15586/jkcvhl.v8i2.175","url":null,"abstract":"<p><p>Lynch syndrome (LS) is an autosomal dominant inherited disorder due to pathogenic variations in the mismatch repair genes, which predisposes to malignancies, most commonly colon and endometrial carcinoma. Muir-Torre syndrome is a subset of LS with cutaneous sebaceous adenoma and keratoacanthoma in addition to the malignancies. Renal cell carcinoma (RCC) in patients with LS is extremely rare. Only 26 cases have been reported and among them, only two cases of papillary RCC. We report a case of synchronous papillary RCC and colonic adenocarcinoma in an 85-year-old male with Lynch/Muir-Torre syndrome. The LS was diagnosed when he presented with multiple sebaceous adenomas and genetic testing showed a pathogenic variant in <i>MSH6</i> mismatch repair gene. A colonoscopy at that time showed multiple tubular adenomas with high-grade dysplasia. He was lost to follow-up and presented with gastrointestinal bleeding after 20 years. A right colonic mass, and a solid mass in the lower pole of the right kidney, was detected by imaging. Right Colectomy showed a T3N0 mucin-producing adenocarcinoma. Right nephrectomy showed a T3a papillary RCC which was microsatellite stable with <i>MSH6</i>, and <i>KRAS</i> mutation. The 36-month follow-up exams showed additional sebaceous neoplasms, and an absence of metastatic carcinoma. Analysis of the reported cases of RCC in LS show clear cell RCC as the most common type. These tumors showed <i>MLH1</i> mutation most commonly, unlike the urothelial malignancies in LS which involve <i>MSH2</i>. Among the 4 cases of RCC with <i>MSH6</i> mutation, three were in females, indicating some gender differences.</p>","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"8 2","pages":"8-19"},"PeriodicalIF":1.6,"publicationDate":"2021-04-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8064920/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38942170","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}
引用次数: 1
Hypoxia-Inducible Factor-2α as a Novel Target in Renal Cell Carcinoma. 缺氧诱导因子-2α作为肾细胞癌的新靶点。
IF 1.6
Journal of Kidney Cancer and VHL Pub Date : 2021-04-07 eCollection Date: 2021-01-01 DOI: 10.15586/jkcvhl.v8i1.170
Won Seok W Choi, Julia Boland, Jianqing Lin
{"title":"Hypoxia-Inducible Factor-2α as a Novel Target in Renal Cell Carcinoma.","authors":"Won Seok W Choi,&nbsp;Julia Boland,&nbsp;Jianqing Lin","doi":"10.15586/jkcvhl.v8i1.170","DOIUrl":"https://doi.org/10.15586/jkcvhl.v8i1.170","url":null,"abstract":"<p><p>Hypoxia-inducible factor (HIF), an important mediator of hypoxia response, is implicated in tumorigenesis in the setting of pseudohypoxia, such as in the inactivation of von Hippel-Lindau tumor suppressor protein (pVHL), leading to development and progression of clear cell renal cell carcinoma (ccRCC). Targeting downstream molecules in HIF pathway, such as vascular endothelial growth factor (VEGF), has led to improvement in clinical outcome for patients with advanced ccRCC, but such therapy thus far has been limited by eventual resistance and treatment failure. Following the discovery of HIF-2α playing a key role in ccRCC carcinogenesis, inhibitors targeting HIF-2α have been developed and have demonstrated encouraging efficacy and safety profile in clinical trials. This review discusses HIF-2α as a promising therapeutic target for ccRCC.</p>","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"8 2","pages":"1-7"},"PeriodicalIF":1.6,"publicationDate":"2021-04-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8033537/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38818775","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}
引用次数: 13
Thrombotic Microangiopathy Associated with Pazopanib in a Kidney Transplant Recipient. 肾移植受者与帕唑帕尼相关的血栓性微血管病变。
IF 1.6
Journal of Kidney Cancer and VHL Pub Date : 2021-03-24 eCollection Date: 2021-01-01 DOI: 10.15586/jkcvhl.v8i1.161
Shabana Kalla, Robert J Ellis, Scott B Campbell, Brian Doucet, Nicole Isbel, Bibiana Tie, Dev Jegatheesan
{"title":"Thrombotic Microangiopathy Associated with Pazopanib in a Kidney Transplant Recipient.","authors":"Shabana Kalla,&nbsp;Robert J Ellis,&nbsp;Scott B Campbell,&nbsp;Brian Doucet,&nbsp;Nicole Isbel,&nbsp;Bibiana Tie,&nbsp;Dev Jegatheesan","doi":"10.15586/jkcvhl.v8i1.161","DOIUrl":"https://doi.org/10.15586/jkcvhl.v8i1.161","url":null,"abstract":"<p><p>Thrombotic microangiopathy (TMA) is characterised by abnormalities in the walls of arterioles and capillaries, precipitated by hereditary or acquired characteristics, and culminating in microvascular thrombosis because of dysregulated complement activity. A number of drugs can precipitate TMA, including vascular endothelial growth factor (VEGF) inhibitors, because of their effects on endothelial repair. Pazopanib is a VEGF inhibitor used for the treatment of renal cell carcinoma (RCC); it is uncommonly associated with TMA. A 52-year-old male, 5 years post his second kidney transplant secondary to immunoglobulin (Ig) A nephropathy, presented with hypertension, fluid overload, and worsening graft function (peak creatinine 275 µmol/L, baseline 130-160 µmol/L) and nephrotic range proteinuria 2 months after commencing pazopanib for metastatic RCC. His maintenance immunosuppression included ciclosporin, mycophenolate, and prednisolone. Haematological parameters were unremarkable. Allograft biopsy demonstrated glomerular and arteriolar changes consistent with chronic active TMA, with overlying features of borderline cellular rejection. He was treated with intravenous methylprednisolone 250 mg for 3 days and commenced on irbesartan 75 mg daily. Drug-induced TMA from pazopanib was suspected, particularly given the documented association with other tyrosine kinase inhibitors (TKIs). In consultation with his medical oncologist, pazopanib was ceased, and an alternate TKI cabozantinib was commenced. Serum creatinine remained <200 µmol/L 3 months after admission. This is the first reported biopsy-proven case of TMA attributed to pazopanib in a kidney transplant recipient. With increasing clinical indications for and availability of TKIs, clinicians need to be aware of their association with TMA events in kidney transplant recipients, who are already susceptible to TMA due to abnormal vasculature, infectious triggers, ischaemia-reperfusion injury, and use of calcineurin inhibitor.</p>","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"8 1","pages":"25-31"},"PeriodicalIF":1.6,"publicationDate":"2021-03-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8017890/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"25598221","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}
引用次数: 6
Germline Pathogenic Variants Identified by Targeted Next-Generation Sequencing of Susceptibility Genes in Pheochromocytoma and Paraganglioma. 嗜铬细胞瘤和副神经节瘤易感基因靶向新一代测序鉴定种系致病变异。
IF 1.6
Journal of Kidney Cancer and VHL Pub Date : 2021-03-13 eCollection Date: 2021-01-01 DOI: 10.15586/jkcvhl.v8i1.171
Sinem Yalcintepe, Hakan Gurkan, Fatma Nur Korkmaz, Selma Demir, Engin Atli, Damla Eker, Hazal Sezginer Guler, Drenusha Zhuri, Emine Ikbal Atli, Semra Ayturk Salt, Mustafa Sahin, Sibel Guldiken
{"title":"Germline Pathogenic Variants Identified by Targeted Next-Generation Sequencing of Susceptibility Genes in Pheochromocytoma and Paraganglioma.","authors":"Sinem Yalcintepe,&nbsp;Hakan Gurkan,&nbsp;Fatma Nur Korkmaz,&nbsp;Selma Demir,&nbsp;Engin Atli,&nbsp;Damla Eker,&nbsp;Hazal Sezginer Guler,&nbsp;Drenusha Zhuri,&nbsp;Emine Ikbal Atli,&nbsp;Semra Ayturk Salt,&nbsp;Mustafa Sahin,&nbsp;Sibel Guldiken","doi":"10.15586/jkcvhl.v8i1.171","DOIUrl":"https://doi.org/10.15586/jkcvhl.v8i1.171","url":null,"abstract":"<p><p>The aim of this study was to evaluate germline variant frequencies of pheochromocytoma and paraganglioma targeted susceptibility genes with next-generation sequencing method. Germline DNA from 75 cases were evaluated with targeted next-generation sequencing on an Illumina NextSeq550 instrument. <i>KIF1B, RET, SDHB, SDHD, TMEM127</i>, and <i>VHL</i> genes were included in the study, and Sanger sequencing was used for verifying the variants. The pathogenic/likely pathogenic variants were in the <i>VHL, RET, SDHB</i>, and <i>SDHD</i> genes, and the diagnosis rate was 24% in this study. Three different novel pathogenic variants were determined in five cases. This is the first study from Turkey, evaluating germline susceptibility genes of pheochromocytoma and paraganglioma with a detection rate of 24% and three novel variants. All patients with pheochromocytoma and paraganglioma need clinical genetic testing with expanded targeted gene panels for higher diagnosis rates.</p>","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"8 1","pages":"19-24"},"PeriodicalIF":1.6,"publicationDate":"2021-03-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7969383/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"25525565","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}
引用次数: 1
Bilateral Single-Stage Nephrectomy for Synchronous Bilateral Renal Cell Carcinoma. 双侧单期肾切除术治疗双侧同步性肾细胞癌。
IF 1.6
Journal of Kidney Cancer and VHL Pub Date : 2021-01-25 eCollection Date: 2021-01-01 DOI: 10.15586/jkcvhl.v8i1.151
Ahmed Kotb, Amer Alaref, David Kisselgoff, Asmaa Ismail, Radu Rozenberg, Nishigandha Burute, Walid Shahrour, Owen Prowse, Hazem Elmanasy
{"title":"Bilateral Single-Stage Nephrectomy for Synchronous Bilateral Renal Cell Carcinoma.","authors":"Ahmed Kotb,&nbsp;Amer Alaref,&nbsp;David Kisselgoff,&nbsp;Asmaa Ismail,&nbsp;Radu Rozenberg,&nbsp;Nishigandha Burute,&nbsp;Walid Shahrour,&nbsp;Owen Prowse,&nbsp;Hazem Elmanasy","doi":"10.15586/jkcvhl.v8i1.151","DOIUrl":"https://doi.org/10.15586/jkcvhl.v8i1.151","url":null,"abstract":"<p><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 dis-concordance in bilateral synchronous RCC.</p>","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"8 1","pages":"7-11"},"PeriodicalIF":1.6,"publicationDate":"2021-01-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7842839/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"25350540","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}
引用次数: 0
Large Retroperitoneal Paraganglioma Associated with Germline Mutation of the Succinate Dehydrogenase Gene. 大腹膜后副神经节瘤与琥珀酸脱氢酶基因的种系突变相关。
IF 1.6
Journal of Kidney Cancer and VHL Pub Date : 2021-01-25 eCollection Date: 2021-01-01 DOI: 10.15586/jkcvhl.v8i1.129
Wen Min Chen, Philip Olson, Rohith Arcot, Huy Nguyen, Faisal Quereshi, Courtney Kokenakes, Michael L Cher
{"title":"Large Retroperitoneal Paraganglioma Associated with Germline Mutation of the Succinate Dehydrogenase Gene.","authors":"Wen Min Chen,&nbsp;Philip Olson,&nbsp;Rohith Arcot,&nbsp;Huy Nguyen,&nbsp;Faisal Quereshi,&nbsp;Courtney Kokenakes,&nbsp;Michael L Cher","doi":"10.15586/jkcvhl.v8i1.129","DOIUrl":"https://doi.org/10.15586/jkcvhl.v8i1.129","url":null,"abstract":"<p><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>","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"8 1","pages":"12-18"},"PeriodicalIF":1.6,"publicationDate":"2021-01-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7842840/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"25350541","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}
引用次数: 2
Epidemiology and Clinicopathological Profile of Renal Cell Carcinoma 肾细胞癌的流行病学和临床病理特征
IF 1.6
Journal of Kidney Cancer and VHL Pub Date : 2021-01-20 DOI: 10.15586/jkcvhl.v8i1.154
Likhiteswer Pallagani, G. Choudhary, H. Pandey, Vijay Kumar Sarma Madurri, Mahendra Tanwar, P. Gupta, N. Shrivastava, Gaurav Baid, M. Rao, A. Nalwa, P. Pareek, S. Misra
{"title":"Epidemiology and Clinicopathological Profile of Renal Cell Carcinoma","authors":"Likhiteswer Pallagani, G. Choudhary, H. Pandey, Vijay Kumar Sarma Madurri, Mahendra Tanwar, P. Gupta, N. Shrivastava, Gaurav Baid, M. Rao, A. Nalwa, P. Pareek, S. Misra","doi":"10.15586/jkcvhl.v8i1.154","DOIUrl":"https://doi.org/10.15586/jkcvhl.v8i1.154","url":null,"abstract":"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.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.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.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.","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"1 1","pages":""},"PeriodicalIF":1.6,"publicationDate":"2021-01-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41788931","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 6
Epidemiology and Clinicopathological Profile of Renal Cell Carcinoma: A Review from Tertiary Care Referral Centre. 肾细胞癌的流行病学和临床病理特征:来自三级保健转诊中心的综述。
IF 1.6
Journal of Kidney Cancer and VHL Pub Date : 2021-01-20 eCollection Date: 2021-01-01 DOI: 10.15586/jkcvhl.2021.154
Likhiteswer Pallagani, Gautam Ram Choudhary, Pandey Himanshu, Vijay K S Madduri, Mahendra Singh, Prateek Gupta, Nikita Shrivastava, Gaurav Baid, Rao Meenakshi, Nalwa Aasma, Puneet Pareek, Misra Sanjeev
{"title":"Epidemiology and Clinicopathological Profile of Renal Cell Carcinoma: A Review from Tertiary Care Referral Centre.","authors":"Likhiteswer Pallagani,&nbsp;Gautam Ram Choudhary,&nbsp;Pandey Himanshu,&nbsp;Vijay K S Madduri,&nbsp;Mahendra Singh,&nbsp;Prateek Gupta,&nbsp;Nikita Shrivastava,&nbsp;Gaurav Baid,&nbsp;Rao Meenakshi,&nbsp;Nalwa Aasma,&nbsp;Puneet Pareek,&nbsp;Misra Sanjeev","doi":"10.15586/jkcvhl.2021.154","DOIUrl":"https://doi.org/10.15586/jkcvhl.2021.154","url":null,"abstract":"<p><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. This was a retrospective study that involved data analysis of records of RCC patients who presented to our institution from April 2016 to February 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. A total 142 RCC patients were included in the study. The median age of presentation was 58 years. Most of the patients (67%) were symptomatic, 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. 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>","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"8 1","pages":"1-6"},"PeriodicalIF":1.6,"publicationDate":"2021-01-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7827726/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"25341788","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}
引用次数: 12
Emerging Therapies for Advanced Clear Cell Renal Cell Carcinoma. 晚期透明细胞肾细胞癌的新疗法。
IF 1.6
Journal of Kidney Cancer and VHL Pub Date : 2020-12-14 eCollection Date: 2020-01-01 DOI: 10.15586/jkcvhl.2020.156
Alexander T Toth, Daniel C Cho
{"title":"Emerging Therapies for Advanced Clear Cell Renal Cell Carcinoma.","authors":"Alexander T Toth,&nbsp;Daniel C Cho","doi":"10.15586/jkcvhl.2020.156","DOIUrl":"https://doi.org/10.15586/jkcvhl.2020.156","url":null,"abstract":"<p><p>Multiple combinational regimens have recently been approved and are now considered the standard of care for patients with advanced clear cell renal cell carcinoma (RCC). Several additional combinational regimens are deep in clinical assessment and are likely to soon join the crowded front-line therapeutic landscape. Most of these regimens are combinations of agents already approved as single-agents in RCC including tyrosine kinase inhibitors (TKI) and immune checkpoint inhibitors. While these new front-line regimens are associated with reliably high response rates and prolonged survival, complete and durable remissions remain limited to a small subset of patients and the vast majority of patients continue to require subsequent therapy. The need for the continued development of novel agents in RCC persists and efforts have focused on agents targeting the molecular biology of clear cell RCC and novel immunotherapies including cytokines. In this review, we discuss the progress in the development of these novel therapies in the context of the evolving standard of care for patients with advanced clear cell RCC.</p>","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"7 4","pages":"17-26"},"PeriodicalIF":1.6,"publicationDate":"2020-12-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7738296/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38751921","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}
引用次数: 6
Adrenal Metastases as Sanctuary Sites in Advanced Renal Cancer. 肾上腺转移是晚期肾癌的避难所。
IF 1.6
Journal of Kidney Cancer and VHL Pub Date : 2020-10-12 eCollection Date: 2020-01-01 DOI: 10.15586/jkcvhl.2020.132
Ulka Vaishampayan, Harsh Shah, Mohammad F Asad, Dongping Shi, Brenda Dickow, Stacey Suisham, Jason Domina, Michael L Cher, Julie Samantray, Hussein D Aoun
{"title":"Adrenal Metastases as Sanctuary Sites in Advanced Renal Cancer.","authors":"Ulka Vaishampayan,&nbsp;Harsh Shah,&nbsp;Mohammad F Asad,&nbsp;Dongping Shi,&nbsp;Brenda Dickow,&nbsp;Stacey Suisham,&nbsp;Jason Domina,&nbsp;Michael L Cher,&nbsp;Julie Samantray,&nbsp;Hussein D Aoun","doi":"10.15586/jkcvhl.2020.132","DOIUrl":"https://doi.org/10.15586/jkcvhl.2020.132","url":null,"abstract":"<p><p>Involvement of the adrenal gland in kidney cancer represents a unique site of metastasis with a distinct clinical course. The cases are typically resistant to immune therapy and need local therapy management. A case series of patients with adrenal metastases was reviewed to highlight the nuances of clinical course and therapy. We reviewed renal cancer carcinoma (RCC) cases with adrenal metastases at Karmanos Cancer Center, Detroit MI. Medical records were reviewed to collect relevant case information. Next-generation sequencing, tumor mutation burden testing, and programmed death ligand biomarkers were evaluated in five cases. Twelve cases were reviewed; all were males with a median age of 49.5 years. Three patients presented with adrenal metastases only and were treated with local therapy. Three received interleukin-2 (IL-2). One patient relapsed with bilateral adrenal lesions after 11 years of remission, post-IL-2 therapy. Five cases received immune checkpoint inhibitor (ICI) and one received antivascular therapy. ICI therapy was followed by ablation of residual adrenal metastases in three patients. Genomic profiling was available in five cases. All were <i>BAP1</i> and PD-L1 negative.Pathogenic mutations in <i>PBRM1, SETD2</i>, and <i>VHL</i> were noted. All patients with residual adrenal metastases responded to antivascular therapies or to local ablation. One patient died 17 years after diagnosis and 11 patients are alive at a median follow-up of 9.5 years. Adrenal metastases in RCC have a distinct clinical course. They can represent a sanctuary site of relapse/residual disease following treatment with immune therapy. Management with local therapy can induce durable remissions. Systemic management with antivascular therapies also demonstrated favorable responses. Further investigation should focus on the unique clinical course and optimal management of adrenal metastases in kidney cancer.</p>","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"7 4","pages":"1-7"},"PeriodicalIF":1.6,"publicationDate":"2020-10-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7597902/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38600040","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}
引用次数: 4
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