Mohamed Sharafeldeen, Wael Sameh, Vahid Mehrnoush, Amer Alaref, Radu Rozenberg, Asmaa Ismail, Hazem Elmansy, Walid Shahrour, Ahmed Zakaria, Osama Elmeslemany, Nishigandha Burute, Anatoly Shuster, Owen Prowse, Ahmed Kotb
{"title":"Partial Nephrectomy for T1b/T2 Renal Mass: An Added Shift from Radical Nephrectomy.","authors":"Mohamed Sharafeldeen, Wael Sameh, Vahid Mehrnoush, Amer Alaref, Radu Rozenberg, Asmaa Ismail, Hazem Elmansy, Walid Shahrour, Ahmed Zakaria, Osama Elmeslemany, Nishigandha Burute, Anatoly Shuster, Owen Prowse, Ahmed Kotb","doi":"10.15586/jkcvhl.v9i4.255","DOIUrl":"https://doi.org/10.15586/jkcvhl.v9i4.255","url":null,"abstract":"<p><p>The aim of our study was to show our short-term experience in managing large renal masses (cT1b/T2) through partial nephrectomy (PN) over the last 3 years. Retrospective data collection for all patients managed by PN for renal masses larger than 4 cm over the last 3 years. Epidemiological data were collected. Surgical data including surgical and ischemic times as well as intra and postoperative complications were collected. Pre- and postoperative estimated glomerular filtration rate (eGFR) data were collected and correlated as well as postoperative complications and recurrence. We could identify 47 patients managed by PN for radiologically confirmed >4 cm renal masses. The mean age of the patients was 55.7 ± 13.4, including 29 males and 18 females. Masses were T1b and T2 in 40 and 7 patients, respectively. The mean tumor size was 6.2 ± 1.5 cm. Using renal nephrometry score; 8, 28, and 11 had low, moderate, and high complexity, respectively. Renal cell carcinoma (RCC) was identified in 42 patients. Five patients out of 42 cancerous cases (12%) had pathological T3 RCC. The mean preoperative and postoperative eGFR were 89.09 ± 12.41 and 88.50 ± 10.50, respectively (P 0.2). The median follow-up was 14 months and within that short time, no patient had evidence for cancer recurrence. PN for large renal masses is safe in experienced hands and should be attempted in a higher percentage of patients, regardless of the tumor complexity. No cancer recurrence or deterioration of renal function was observed within our short-term follow-up.</p>","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":" ","pages":"1-5"},"PeriodicalIF":1.6,"publicationDate":"2022-10-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9550639/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40657815","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}
Iraklis C Mitsogiannis, Maria Mitsogianni, Maria Papathanassiou, Maria Anagnostou, Ioannis Tamposis, Lampros Mitrakas, Maria Samara, Vassilios Tzortzis, Panagiotis J Vlachostergios
{"title":"Current Options for Second-Line Systemic Therapy in Metastatic Renal Cell Carcinoma.","authors":"Iraklis C Mitsogiannis, Maria Mitsogianni, Maria Papathanassiou, Maria Anagnostou, Ioannis Tamposis, Lampros Mitrakas, Maria Samara, Vassilios Tzortzis, Panagiotis J Vlachostergios","doi":"10.15586/jkcvhl.v9i3.243","DOIUrl":"https://doi.org/10.15586/jkcvhl.v9i3.243","url":null,"abstract":"<p><p>Standard systemic therapy of advanced renal cell carcinoma (RCC) involves targeting angiogenesis, mainly through tyrosine kinase inhibitors (TKI) against the vascular endothelial growth factor receptor (VEGFR) pathway and targeting the immune checkpoints, namely, programmed death-1 (PD-1) or its ligand (PD-L1), and cytotoxic T-lymphocyte-associated protein 4 (CTLA4). With current strategies of combining these two approaches in the front-line setting, less is known about optimal selection of therapy upon development of resistance in the second and later lines of treatment for progressive disease. This review discusses currently available therapeutic options in patients who have progressive RCC after prior treatment with double immune check-point inhibitors (ICIs) or ICI-TKI combinations.</p>","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":" ","pages":"29-40"},"PeriodicalIF":1.6,"publicationDate":"2022-09-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9551369/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40656250","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}
Aileen Arevalo, Neal Patel, Peter Muraki, Shinji Ohtake, Gennady Bratslavsky, Chandra Clark, Joshua Mann, Othon Iliopoulos, Eric Jonasch, Ramaprasad Srinivasan, Brian Shuch
{"title":"Understanding the Impact of Belzutifan on Treatment Strategies for Patients with VHL.","authors":"Aileen Arevalo, Neal Patel, Peter Muraki, Shinji Ohtake, Gennady Bratslavsky, Chandra Clark, Joshua Mann, Othon Iliopoulos, Eric Jonasch, Ramaprasad Srinivasan, Brian Shuch","doi":"10.15586/jkcvhl.v9i3.245","DOIUrl":"https://doi.org/10.15586/jkcvhl.v9i3.245","url":null,"abstract":"<p><p>Belzutifan was recently approved for the management of Von Hippel-Lindau disease (VHL). Given the morbidity of recurrent treatment, systemic therapy to reduce or eliminate the need for surgery has been long-awaited. Herein, we sought to gain insight about future utilization by surveying VHL kidney cancer experts in the United States. A survey developed by members of the VHL Alliance (VHLA) Clinical Advisory Council was distributed to kidney cancer providers at VHLA and National Comprehensive Cancer Network (NCCN) centers. Surveys were administered on a secure web-based platform. A total of 60 respondents from 29 institutions participated. Urologists (50%) and medical oncologists (43%) represented the majority of participants. The majority (98%) of respondents anticipated that belzutifan's approval would significantly change the current treatment landscape. Most reported that therapy should be continuous (76%). There was a difference in willingness to prescribe belzutifan by specialty (38% of urologists vs 91% of medical oncologists (P = 0.02)). In individuals with renal tumors <3 cm, 36% would still recommend surveillance, while 36% would initiate belzutifan to prevent growth. In those with multifocal renal lesions and growth of a solitary tumor on belzutifan, 50% would proceed with only treatment of that site. In conclusion, VHL kidney cancer specialists anticipate a paradigm shift with the approval of belzutifan. Provider roles may change with movement away from surgical management. Opinions on treatment indications, such as when to initiate therapy and how to best salvage, vary widely and therefore collaborative efforts among experts may assist in the development of new clinical guidelines.</p>","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":" ","pages":"41-46"},"PeriodicalIF":1.6,"publicationDate":"2022-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9551368/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40656249","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}
Ali Eslahi, Shahryar Zeighami, Faisal Ahmed, Seyed Hossein Hosseini, Bahareh Ebrahimi, Mohammad Hossein Anbardar
{"title":"Synchronized Laparoscopic Bilateral Adrenalectomy for Pheochromocytoma in Multiple Endocrine Neoplasia Syndrome: A Case Report.","authors":"Ali Eslahi, Shahryar Zeighami, Faisal Ahmed, Seyed Hossein Hosseini, Bahareh Ebrahimi, Mohammad Hossein Anbardar","doi":"10.15586/jkcvhl.v9i3.239","DOIUrl":"https://doi.org/10.15586/jkcvhl.v9i3.239","url":null,"abstract":"<p><p>Pheochromocytomas are tumors producing catecholamines that arise from chromaffin cells in the adrenal medulla. They are usually benign in multiple endocrine neoplasia type 2 (MEN2) syndrome, but they tend to present bilaterally in 50-80% of the patients. Few researchers have reported success with simultaneous laparoscopic bilateral adrenalectomy. Hence, we report a 48-year-old woman who presented with a panic attack, headache, and abdominal discomfort that had started 10 years ago. The computed tomography (CT) scan showed a large bilateral cystic lesion in both adrenal glands in favor of pheochromocytomas (30 × 22 mm and 18 × 15 mm on the right side and 40 × 33 mm and 35 × 28 mm on the left side). The patient underwent bilateral laparoscopic adrenalectomy without intraoperative or postoperative complications. The total blood loss was 50 cc, and the operative time was 4 h. The histopathology of the specimen revealed pheochromocytomas of adrenal masses. In conclusion, our case demonstrates that synchronized laparoscopic bilateral adrenalectomy can be a safe and feasible treatment option for pheochromocytomas in MEN2 patients.</p>","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"9 3","pages":"24-28"},"PeriodicalIF":1.6,"publicationDate":"2022-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9463070/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"33467023","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}
Zev Leopold, Rachel Passarelli, Mark Mikhail, Alexandra Tabakin, Kevin Chua, Ronald D Ennis, John Nosher, Eric A Singer
{"title":"Modern Management of Localized Renal Cell Carcinoma- Is Ablation Part of the Equation?","authors":"Zev Leopold, Rachel Passarelli, Mark Mikhail, Alexandra Tabakin, Kevin Chua, Ronald D Ennis, John Nosher, Eric A Singer","doi":"10.15586/jkcvhl.v9i3.233","DOIUrl":"https://doi.org/10.15586/jkcvhl.v9i3.233","url":null,"abstract":"<p><p>While the gold-standard for management of localized renal cell carcinoma (RCC) is partial nephrectomy, recent ablative strategies are emerging as alternatives with comparable rates of complications and oncologic outcomes. Thermal ablation, in the form of radiofrequency ablation and cryoablation, is being increasingly accepted by professional societies, and is particularly recommended in patients with a significant comorbidity burden, renal impairment, old age, or in those unwilling to undergo surgery. Maturation of long-term oncologic outcomes has further allowed increased confidence in these management strategies. New and exciting ablation technologies such as microwave ablation, stereotactic body radiotherapy, and irreversible electroporation are emerging. In this article, we review the existing management options for localized RCC, with specific focus on the oncologic outcomes associated with the various ablation modalities.</p>","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":" ","pages":"5-23"},"PeriodicalIF":1.6,"publicationDate":"2022-08-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9396960/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40346733","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}
Mohamed Sharafeldeen, Vahid Mehrnoush, Asmaa Ismail, Ahmed Zakaria, Hazem Elmansy, Walid Shahrour, Owen Prowse, Ahmed Kotb
{"title":"Successful Surgical Management of Locally Advanced Renal Cell Carcinoma Invading Spleen and Pancreas.","authors":"Mohamed Sharafeldeen, Vahid Mehrnoush, Asmaa Ismail, Ahmed Zakaria, Hazem Elmansy, Walid Shahrour, Owen Prowse, Ahmed Kotb","doi":"10.15586/jkcvhl.v9i3.231","DOIUrl":"https://doi.org/10.15586/jkcvhl.v9i3.231","url":null,"abstract":"<p><p>Over the last two decades, the treatment of metastatic RCC has changed significantly, and the role of surgery is being debated. A 50-year-old man presented with pain in his left loin. An ultrasound, followed by a CT scan, revealed a 17.5 cm left renal mass invading the left suprarenal gland, spleen, and pancreatic tail. Radical nephrectomy through chevron incision under epidural block with general anesthesia was performed. The entire mass was removed en bloc. The estimated blood loss was 300 mL, and no blood transfusions were performed. The operation took approximately 2 h. Histological examination revealed clear cell renal carcinoma with extension into the spleen, pancreatic tail, and diaphragmatic fibers with negative resection margin. The patient discharged after a 3-day uneventful hospital stay. Aggressive surgical removal of a locally invasive renal cell carcinoma is feasible and should be considered in patients with good performance status and no or minimal distant metastases.</p>","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":" ","pages":"1-4"},"PeriodicalIF":1.6,"publicationDate":"2022-08-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9396959/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40346732","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}
Catarina Machado, Maria Sofia Quental, J. R. Brandão, M. Silva‐Ramos
{"title":"Hereditary Leiomyomatosis and Renal Cell Cancer","authors":"Catarina Machado, Maria Sofia Quental, J. R. Brandão, M. Silva‐Ramos","doi":"10.1007/978-3-642-16483-5_2682","DOIUrl":"https://doi.org/10.1007/978-3-642-16483-5_2682","url":null,"abstract":"","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"1 1","pages":""},"PeriodicalIF":1.6,"publicationDate":"2022-05-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43503134","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}
Tavares Catarina, Maria Sofia Quental, José Ricardo Brandão, Miguel Silva-Ramos
{"title":"Hereditary Leiomyomatosis and Renal Cell Cancer-Recognizing Patterns May Save Lives.","authors":"Tavares Catarina, Maria Sofia Quental, José Ricardo Brandão, Miguel Silva-Ramos","doi":"10.15586/jkcvhl.v9i2.222","DOIUrl":"https://doi.org/10.15586/jkcvhl.v9i2.222","url":null,"abstract":"<p><p>In 2001, Finish investigators described a rare familiar syndrome characterized by an inherited susceptibility to cutaneous leiomyomas, uterine leiomyomas, and renal cell carcinoma (RCC). Hereditary leiomyomatosis and renal cell cancer (HLRCC) is now linked to a germline mutation in the <i>fumarate hydratase</i> (<i>FH</i>) gene that encodes a Krebs cycle enzyme, transforming fumarate to malate. The accumulation of fumarate, an oncometabolite, promotes tumorigenesis. We present a case of a 41-year-old female diagnosed with HLRCC after a radical nephrectomy due to renal cell cancer. Genetic analyses confirmed a novel <i>FH</i> mutation. Close follow-up allowed for a precocious diagnosis of a metachronous renal tumor and later a hepatic metastasis. Her family was also counseled and offered genetic testing. As observed in this case, the diagnosis of HLRCC is of paramount importance for patients and their families: there is a 15% cumulative lifetime risk of developing RCC, which frequently occurs in young patients and metastasizes at an early stage. Implementing a regular follow-up with adequate imaging examinations may help save lives.</p>","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":" ","pages":"27-31"},"PeriodicalIF":1.6,"publicationDate":"2022-05-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9430984/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40368314","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}
Ellen M Cahill, Alexandra Tabakin, Brian Shinder, Mark Bramwit, Biren Saraiya, Xinyang Xu, Cristo G Salazar, Zhongren Zhou, Eric A Singer
{"title":"Renal Cell Carcinoma with Cardiac Metastases: A Case Report and Review of the Literature.","authors":"Ellen M Cahill, Alexandra Tabakin, Brian Shinder, Mark Bramwit, Biren Saraiya, Xinyang Xu, Cristo G Salazar, Zhongren Zhou, Eric A Singer","doi":"10.15586/jkcvhl.v9i2.229","DOIUrl":"https://doi.org/10.15586/jkcvhl.v9i2.229","url":null,"abstract":"<p><p>Cardiac metastases from renal cell carcinoma (RCC) are very rare. We describe the case of a woman with RCC with cardiac metastases involving the entire right atrium, penetrating through the myocardium, with extension into the tricuspid valve and right ventricle. This report highlights the unique challenge of the diagnosis and treatment of cardiac metastases in RCC.</p>","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":" ","pages":"32-38"},"PeriodicalIF":1.6,"publicationDate":"2022-05-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9431006/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40368315","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}
Sho Yoshitake, Brian M Shinder, Kevin Dazen, Colton Smith, Tina M Mayer, Evita Sadimin, Eric A Singer
{"title":"Renal Cell Carcinoma with Testicular Metastases: A Case Report and Review of the Literature.","authors":"Sho Yoshitake, Brian M Shinder, Kevin Dazen, Colton Smith, Tina M Mayer, Evita Sadimin, Eric A Singer","doi":"10.15586/jkcvhl.v9i2.212","DOIUrl":"https://doi.org/10.15586/jkcvhl.v9i2.212","url":null,"abstract":"<p><p>Renal cell carcinoma (RCC) metastases to the testicle are an extremely rare clinical entity. Here, we describe the case of a man with metastatic RCC who developed a new testicular mass. Pathologic analysis after surgical removal of this testicle confirmed the diagnosis of metastatic RCC. This report highlights the unique diagnostic and therapeutic challenges associated with such a disease process.</p>","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":" ","pages":"22-26"},"PeriodicalIF":1.6,"publicationDate":"2022-05-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9430971/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40368312","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}