Christopher L Sudduth, Anthony Castagno, Peter Maggs
{"title":"Delayed Cardiac Metastasis from Renal Cell Carcinoma Caused by <i>VHL</i> Mutation.","authors":"Christopher L Sudduth, Anthony Castagno, Peter Maggs","doi":"10.15586/jkcvhl.v10i1.258","DOIUrl":"https://doi.org/10.15586/jkcvhl.v10i1.258","url":null,"abstract":"<p><p>Cardiac metastasis caused by renal cell carcinoma (RCC) without vena caval involvement is rare. No mutation has been associated with this unique phenotype. A 77-year-old male presented to our clinic with a symptomatic right ventricular mass after nephrectomy for clear cell RCC (ccRCC). The mass was resected, and metastatic disease was confirmed. Targeted exon sequencing identified a <i>VHL</i> mutation (c.494T > G, p.V165G) in the resected specimen. While more than half of ccRCC cases are associated with <i>VHL</i> mutations, this case is the first to show the association between delayed, isolated cardiac metastasis and <i>VHL</i> V165G mutation. The phenotype presented 12 years after nephrectomy and localized to the right ventricular apex. Further genomic characterization of cases with cardiac metastases may provide clues regarding unique mutations noted. Patients exhibiting delayed spread of RCC to the heart must be screened for this mutation.</p>","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"10 1","pages":"15-18"},"PeriodicalIF":1.6,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9922489/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10755237","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}
Natarajan Ramya, Murhekar Kanchan, Raja Anand, Sundersingh Shirley
{"title":"A Case Report: Mucinous Tubular and Spindle Cell Carcinoma of Kidney with Spindle Cell Predominance Mimicking Mesenchymal Tumour.","authors":"Natarajan Ramya, Murhekar Kanchan, Raja Anand, Sundersingh Shirley","doi":"10.15586/jkcvhl.v9i4.203","DOIUrl":"https://doi.org/10.15586/jkcvhl.v9i4.203","url":null,"abstract":"<p><p>Mucinous tubular and spindle cell carcinoma (MTSCC) of kidney is a rare variant of renal cell carcinoma which was first described in the 2004 World Health Organization classification of tumours of the kidney. Morphologically, MTSCC is composed of tubules merging with bland-appearing spindle cells in a myxoid/mucinous stroma. Diverse morphological patterns have been reported in MTSCC; however, a spindle cell predominant morphology mimicking a mesenchymal tumour is rare and only two cases have been reported so far. We report a case of MTSCC with spindle cell predominance in kidney which was a diagnostic challenge. Though MTSCC usually shows an indolent course, there have been cases showing aggressive behaviour even with bland morphology. Hence, a thorough histopathological evaluation with ancillary studies are required to differentiate spindle cell predominant MTSCC from its mimics. Our case was a 40-year-old female who was incidentally found to have a well-defined hypodense lesion measuring around 2 cm in the upper pole of the right kidney. Right partial nephrectomy was performed which showed a 2.7 × 2.5 × 2 cm well-defined grey tan tumour without necrosis or haemorrhage, limited to kidney. Histopathological examination showed sheets of bland-appearing spindle cells mimicking a mesenchymal tumour. The tumour was extensively sampled, revealing small foci of tubule formation and mucinous stroma. Tumour cells were positive for CK7, AMACR, and PAX8. A final diagnosis of MTSCC was made. Hereby, we discuss ways of differentiating MTSCC from other spindle cell tumours of the kidney.</p>","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":" ","pages":"10-13"},"PeriodicalIF":1.6,"publicationDate":"2022-10-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9634216/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40468529","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":"Mucinous Tubular and Spindle Cell Renal Cell Carcinoma (MTSC-RCC) with an Unusual Presentation in a Child.","authors":"Kanika Sharma, Anjan Dhua, Sandeep Agarwala, Seema Kaushal","doi":"10.15586/jkcvhl.v9i4.223","DOIUrl":"https://doi.org/10.15586/jkcvhl.v9i4.223","url":null,"abstract":"<p><p>Mucinous tubular and spindle cell renal cell carcinoma (MTSC-RCC) is a rare but favorable variant of renal cell carcinoma, predominantly found in adults. Complete surgical excision is the treatment of choice. We are reporting an intriguing case of bilateral MTSC-RCC in a 13-year-old-boy with rapid disease progression, leading to metastatic disease and subsequent demise of the child.</p>","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":" ","pages":"6-9"},"PeriodicalIF":1.6,"publicationDate":"2022-10-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9629142/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40468528","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, 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}