Journal of Kidney Cancer and VHL最新文献

{"title":"Epithelial Predominant Wilms Tumor in an Adult Patient: Case Report and Literature Review.","authors":"Sofia Chapman, Benjamin Lichtbroun, Hiren Patel, Sai Krishnaraya Doppalapudi, Hatim Thaker, Colton Smith, Cristo Guardado Salazar, Scott Moerdler, Saum Ghodoussipour","doi":"10.15586/jkcvhl.v11i3.329","DOIUrl":"10.15586/jkcvhl.v11i3.329","url":null,"abstract":"<p><p>Although rare in adults, Wilms tumor is the most common pediatric renal tumor. Treatment typically involves radical nephrectomy followed by adjuvant chemotherapy or radiation, although outcomes differ between children and adults which may be due to challenges in accurately diagnosing these patients. In this article, we present a case report of an adult patient with Jeune syndrome and multiple urologic abnormalities who underwent radical nephrectomy for a large renal mass and was subsequently diagnosed with an epithelial predominant Wilms tumor. Epithelial predominant Wilms tumor may have distinct origins from other Wilms tumor histological subtypes and may incur better outcomes. Herein, we discuss the literature surrounding this rare entity as well as the anticipated treatment course.</p>","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"11 3","pages":"33-39"},"PeriodicalIF":1.9,"publicationDate":"2024-08-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11325451/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141989174","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":"Comparison of Trifecta and Pentafecta Outcomes across 3 Surgical Modalities of Partial Nephrectomy (PN) - Open, Lap, and Robotic.","authors":"Hiranya Deka, N Mallikarjunarao Medam, Ginil Kumar P, Vishnu P, Manav Gideon, Achuth Ajith Kumar, Yensani Prashanth Reddy, Shivraj Barath Kumar","doi":"10.15586/jkcvhl.v11i3.308","DOIUrl":"10.15586/jkcvhl.v11i3.308","url":null,"abstract":"<p><p>Renal cell carcinoma (RCC) is the most common solid tumor in the kidney (90%), accounting for about 3% of all cancers in adults. Partial nephrectomy (PN) is the surgical procedure primarily used for the treatment of localized kidney tumors. Two commonly used terms to describe the complexity and success of a partial nephrectomy procedure are \"trifecta\" and \"pentafecta.\" Trifecta is defined as Warm ischemia time (WIT) ≤ 25min or Cold ischemia time (CIT) ≤ 60min, Negative surgical margin (NSM), and no perioperative Clavien-Dindo complications (CDC) of Gr 3 or more [8], whereas pentafecta is defined as trifecta plus >90% preservation of e-Glomerular filtration rate (GFR) and no increase in chronic kidney disease (CKD) stage at 12-months post-operative period. We retrospectively analyzed all patients who underwent partial nephrectomy at a single high-volume tertiary centre, from 2012 to 2020. We included patients who underwent partial nephrectomy by any of the three routes including open (OPN), laparoscopic (LPN), or robotic-assisted (RPN), and in which the follow-up data was available. We compared the trifecta and pentafecta outcomes across the three surgical modalities. We had a total of 183 patients in our study. Twenty-nine percent (53 patients) underwent open surgery, 12.6% (23 patients) underwent laparoscopic surgery and 58.5% (107) underwent robotic assisted surgery. The number of patients who fell under the low risk category in the RENAL scoring system were 70(38.3%), intermediate risk 79 (43.2%) and high risk 34 (18.6%). In the high risk RENAL score group, trifecta was achieved in 5 (50%) patients in OPN, 1(50%) in LPN and 7(31.8%) in RPN with no statistically significant difference (p = 0.581) whereas pentafecta was achieved in 3 (30%) patients in OPN, 1 (50%) in LPN and 7 (31.8%) in RPN with no statistically significant difference (0.855). In the overall cohort, mean WIT, mean hospital stay and mean EBL were higher in OPN as compared to LPN and RPN which was statistically significant (p < 0.001), whereas there was no statistical difference in mean operative time between the three modalities (p = 0.580). Renal tumors can be safely treated by RPN or LPN with lesser morbidity as compared to OPN. Trifecta and Pentafecta outcomes had no significant difference among OPN, LPN, and RPN. RPN and LPN may be considered feasible and safe surgical approaches ensuring good functional outcomes.</p>","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"11 3","pages":"27-32"},"PeriodicalIF":1.9,"publicationDate":"2024-08-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11316447/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141917661","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":"Clinical T1a Renal Cell Carcinoma with Solitary Diaphragmatic Metastasis in a Patient with von Hippel-Lindau Disease.","authors":"Tadataka Hirai, Mayu Uka, Toshihiro Iguchi, Kazuya Yasui, Takahiro Kawabata, Noriyuki Umakoshi, Koji Tomita, Yusuke Matsui, Yasuyuki Kobayashi, Motoo Araki, Takao Hiraki","doi":"10.15586/jkcvhl.v11i3.342","DOIUrl":"10.15586/jkcvhl.v11i3.342","url":null,"abstract":"<p><p>We report the case of a 38-year-old man with two von Hippel-Lindau disease-associated T1a renal cell carcinomas (RCCs) (<2 cm in diameter) which developed into a 2.5-cm solitary diaphragmatic metastatic tumor. After diagnosis using percutaneous biopsy, the diaphragmatic metastasis and two RCCs were treated by laparoscopic resection and percutaneous cryoablation, respectively. One year after treatment, the patient survived without local recurrence or distant metastasis. This report describes a rare case of RCC metastasis in VHL disease and its treatment.</p>","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"11 3","pages":"23-26"},"PeriodicalIF":1.9,"publicationDate":"2024-08-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11299040/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141894562","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":"Clinical, Genomic, and Transcriptomic Characteristics of Patients with Metastatic Renal Cell Carcinoma Who Developed Thromboembolic Events.","authors":"Gliceida Galarza Fortuna, Beverly Chigarira, Vinay Mathew Thomas, Kamal Kant Sahu, Shruti Adidam Kumar, Nishita Tripathi, Nicolas Sayegh, Neeraj Agarwal, Umang Swami, Benjamin L Maughan, Haoran Li","doi":"10.15586/jkcvhl.v11i3.319","DOIUrl":"10.15586/jkcvhl.v11i3.319","url":null,"abstract":"<p><p>Thromboembolic events (TE) are a common complication in patients with metastatic renal cell carcinoma (mRCC) and are associated with poorer clinical outcomes. However, the incidence of TE and clinical and genomic characteristics of patients with mRCC who develop this complication are poorly understood. Herein, we describe the incidence and clinical features of patients with mRCC with or without TE at our institution, and examine their association with the underlying genomic and transcriptomic characteristics of the tumor. This retrospective study included all consecutive cases of mRCC seen at our institution. A CLIA-certified lab performed tumor genomics and transcriptomics. Patients were classified based on the presence of a TE within the first year of diagnosis. Three hundred and seventy patients with mRCC were included in the study. TE was seen in 11% (42) of the patients. Patients with favorable International mRCC Database Consortium (IMDC) risk were less likely to develop a TE. In contrast, patients receiving combination treatment with a tyrosine kinase inhibitor (TKI) and an immune checkpoint inhibitor were more likely to develop a TE. No difference in overall survival among patients with or without TE was observed (52 vs. 55 months; HR 0.85, 95% CI 0.5574-1.293, p = 0.24). The most upregulated pathways in mRCC with TEs versus those without were the xenobiotic metabolism and mTORC1 signaling pathways. Our findings suggest potential biomarkers that, after external validation, could be used to better select patients who would benefit from prophylactic anticoagulation.</p>","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"11 3","pages":"13-22"},"PeriodicalIF":1.9,"publicationDate":"2024-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11296887/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141890371","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":"Nephrectomies in Saudi Arabia: A Comprehensive Analysis of Outcomes from a High-Volume Minimally Invasive Surgery Center.","authors":"Ahmed Alasker, Turki Rashed Alnafisah, Areez Shafqat, Belal Nedal Sabbah, Marwan Alaswad, Mohammad Alghafees, Abdullah Alhaider, Abdulrahman Alsayyari, Naif Althonayan, Mohammed Al-Otaibi, Salman Bin Ofisan, Mohammed Ghazi Alharbi, Bader Alsaikhan, Abdullah Al-Khayal","doi":"10.15586/jkcvhl.v11i3.332","DOIUrl":"10.15586/jkcvhl.v11i3.332","url":null,"abstract":"<p><p>Literature reporting on the outcomes of the different procedures of nephrectomies (open vs laparoscopic vs robotic) in Saudi Arabia remains limited. Compare surgical and oncological outcomes between open and minimally invasive nephrectomies. A retrospective cohort study. The present study included all adult patients who underwent nephrectomies between January 1, 2015 and January 31, 2023. We collected demographic, preoperative, intraoperative, and postoperative data on 408 adult cancer patients who underwent nephrectomies at our center between January 2015 and January 2023. Statistical differences were calculated between procedure types. Overall survival was calculated using Kaplan-Meier curves with log-rank tests. P<0.05 was considered statistically significant. Measures of operative success (intraoperative blood loss, intraoperative and postoperative complications, and hospital stay) and oncological outcomes (local recurrence, metastatic progression, and chemotherapy use) between different procedure and nephrectomy types for cancer patients. A total of 408 cancer patients underwent nephrectomies. In cancer patients, open nephrectomy was associated with significantly higher intraoperative blood loss (p<0.001), incidence of blood transfusions (p<0.001), hospital stay (p<0.001), intraoperative complications (p=0.027 and p=0.001, respectively), local recurrence (p<0.001), metastatic progression (p=0.001), and chemotherapy (p=0.001) than minimally invasive surgery, but survival differences across procedure types were not statistically significant (log-rank p-value = 0.054). Regarding nephrectomy type, significant differences were observed in tumor size (p < 0.001), initial procedure type (p<0.001), operation time (p<0.001), blood transfusion (p=0.033), length of hospital stay (p=0.004), intraoperative complications (p=0.020), postoperative complications (p=0.025), Clavien classification (p=0.003), mortality (p=0.022), metastatic progression (p<0.001), and chemotherapy use (p=0.001) between simple/total nephrectomy, radical nephrectomy (RN), partial nephrectomy (PN), and nephroureterectomy. Survival differences between the four nephrectomy types were statistically significant (log-rank p value = 0.001). Minimally invasive nephrectomies reduce inpatient morbidity while conferring equivalent oncological and surgical outcomes.</p>","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"11 3","pages":"1-12"},"PeriodicalIF":1.9,"publicationDate":"2024-07-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11261389/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141752982","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":"Preoperative Super-Selective Embolization versus \"On-Clamp\" Laparoscopic Partial Nephrectomy for T1 Renal Tumors- A Prospective Randomized Study.","authors":"Vivek Kumar Singh, Debanga Sarma, Sushant Agarwal, Puskal Kumar Bagchi, Mandeep Phukan, Nabajeet Das, Sasanka Kumar Barua","doi":"10.15586/jkcvhl.v11i2.328","DOIUrl":"10.15586/jkcvhl.v11i2.328","url":null,"abstract":"<p><p>To analyze and compare the intraoperative and post-operative outcomes of \"on-clamp\" laparoscopic partial nephrectomy (LPN) with \"preoperative super-selective angioembolization\" before LPN. This randomized clinical study was conducted at Gauhati Medical College Hospital, Guwahati, India, between November 2021 and November 2023. Adult patients of either gender diagnosed with T1 renal tumors were included in the study. All patients underwent diethylenetriamine pentaacetate scan preoperatively and at 1-month follow-up. The patients were randomized using a parallel group design with an allocation ratio of 1:1 to receive either preoperative angioembolization followed by LPN or conventional \"on-clamp\" LPN. Demographic and baseline parameters were recorded along with pre- and post-operative data. There was no significant difference between the two groups in terms of age (P = 0.11), gender distribution (P = 0.32), body mass index (P = 0.43), preoperative hemoglobin (P = 0.34), and preoperative estimated glomerular filtration rate (eGFR; P = 0.64). One patient in the embolization group required radical nephrectomy because of accidental backflow of glue into the renal artery during embolization whereas four patients required clamping due to inadequate embolization. Preoperative super-selective embolization yielded significantly less blood loss, compared to \"on-clamp\" LPN (145 [50.76 mL] vs. 261 [66.12 mL], P < 0.01). There was no significant difference between post-operative eGFR (at 1 month) between the two groups (P = 0.71). Preoperative embolization offers improved outcomes in the dissection plane, total operative time, and blood loss, compared to conventional \"on-clamp\" LPN but has no significant effect on change in eGFR.</p>","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"11 2","pages":"18-26"},"PeriodicalIF":1.6,"publicationDate":"2024-05-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11126868/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141155343","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":"Native Kidney Renal Cell Carcinoma in Renal Allograft Transplant Patients - Our Experience.","authors":"Pavan Survase, Shashank Agrawal, Abhishek Singh, Ravindra Sabnes, Mahesh Desai","doi":"10.15586/jkcvhl.v11i2.283","DOIUrl":"10.15586/jkcvhl.v11i2.283","url":null,"abstract":"<p><p>The immunosuppression administered to renal transplant recipients to safeguard renal function elevates their susceptibility to renal cancer, which is estimated to be 15 times higher than in the general population. The current study aimed to analyze various aspects of native kidney renal cell carcinoma (RCC) in renal transplant recipients. This study involved a retrospective analysis of 11 patients who underwent nephrectomy for RCC in native kidneys among renal transplant recipients at our institution since 1992. Our institutional incidence was 0.4%. Median age at presentation was 57 (49-60) years. The ratio of male: female was 10:1. Most patients were asymptomatic at presentation and native kidney disease before transplantation was undetermined. In our study, the median time interval between diagnosis of RCC and transplant was 9.1 (8.4-11.2) years. All patients underwent native kidney nephrectomy. Clear cell type was more common than papillary type, 3.5 (2.5-4.2). Ten patients were diagnosed with stage I disease and one patient had stage IV disease. Fuhrman nuclear grading revealed low grades in nine patients and three patients had Grade 3. Immunosuppressive therapy modification was done in nine patients. Meticulous follow-up of renal transplant patients is essential for earlier diagnosis and appropriate treatment of native kidney RCC in transplant recipients. Authors recommend every year follow-up in transplant recipients with special emphasis on ultrasound of native kidney.</p>","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"11 2","pages":"7-11"},"PeriodicalIF":1.6,"publicationDate":"2024-05-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11109759/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141080475","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 Survival Outcomes of the Metastatic Nonclear Cell Renal Cell Carcinoma in the Immunotherapy Era: Princess Margaret Cancer Centre Experience.","authors":"Esmail Al-Ezzi, Abhenil Mittal, Zachary W Veitch, Amer Zahralliyali, Nely Mercy Diaz Mejia, Osama Abdeljalil, Husam Alqaisi, Vikaash Kumar, Aaron R Hansen, Nazanin Fallah-Rad, Srikala S Sridhar","doi":"10.15586/jkcvhl.v11i1.307","DOIUrl":"10.15586/jkcvhl.v11i1.307","url":null,"abstract":"<p><p>Immunotherapy (IO) with or without targeted therapy (TT) is the standard treatment for patients with metastatic clear cell renal cell carcinoma (RCC). The evidence supporting their use in metastatic nonclear cell renal cell carcinoma (nccRCC) subtypes is based on small prospective trials and retrospective analyses. Here, we report survival outcomes for patients with metastatic nccRCC treated with IO and/or TT at the Princess Margaret Cancer Centre, Toronto, ON, Canada. Demographics, disease characteristics, and survival outcomes were collected retrospectively. Overall (OS), progression-free survival (PFS), and objective response rates (ORR) were calculated. We identified 69 patients with metastatic nccRCC treated with IO and/or TT as the first-line treatment, and 36 (52.1%) patients as the second-line treatment. Median OS of the first line IO recipients (n = 12) and non-IO recipients (n = 57) was not reached (NR) and 17.2 months (95% confidence interval (95% CI): 7.3-27.0; P = 0.23), respectively. Median PFS of first-line IO recipients and non-IO recipients was NR and 4.7 months (95% CI: 3.7-5.6; P = 0.019), respectively. The ORR of IO recipients versus non-IO recipients was 50% versus 12.3% (P = 0.007). Median OS of the second-line IO recipients (n = 8) and non-IO recipients (n = 28) was NR and 6.3 months (95% CI: 3.2-9.3; P = 0.003), respectively. Median PFS of second-line IO recipients and non-IO recipients was 4.8 months (95% CI: 2.7-6.8) and 2.8 months (95% CI: 1.8-3.7; P = 0.014), respectively. ORR of IO recipients and non-IO recipients was 37.5% and 3.5%, respectively; P = 0.028. While the number of patients included in our retrospective review was small, our analysis suggested that patients with nccRCC have improved survival outcomes with IO treatment. Validation of prospective dataset is required before widespread clinical utilization.</p>","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"11 1","pages":"41-48"},"PeriodicalIF":1.6,"publicationDate":"2024-03-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10915653/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140050668","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":"Inoperable Renal Malignant Glomus Tumor, the answers for all the \"W's\"?","authors":"Pushkala Surianarayanan, Arun Ramdas Menon, Shriley Sundersingh, Anand Raja","doi":"10.15586/jkcvhl.v11i1.298","DOIUrl":"10.15586/jkcvhl.v11i1.298","url":null,"abstract":"<p><p>Glomus tumor, arising from glomus bodies (specialized neurovascular structures involved in thermoregulation), commonly occurs in extremities and rarely in viscera. The spectrum of glomus tumors range from benign tumors to tumors with uncertain malignant potential to tumors of the malignant subtype. A vast majority of visceral glomus tumors are benign. Most common visceral tumors arise in the gastrointestinal tract. Glomus tumors of the kidney are a rare entity of which malignant glomus tumors are exceedingly rare. The index patients in the existing case reports were middle-aged males. We report our experience with malignant glomus tumor of the left kidney in a 60-year-old female, with computed tomography (CT) showing involvement of renal vein and inferior vena cava (IVC). Percutaneous biopsy was performed as imaging did not conform to the appearance of a conventional renal tumor and was reported as malignant glomus tumor after immunohistochemistry. After informed decision, the patient and family elected to proceed with surgery. However, intraoperatively, the left renal mass was found to infiltrate the pancreas, duodenum, aorta, and root of the colonic mesentery due to which surgery was aborted. Biopsy obtained intraoperatively again confirmed diagnosis of left renal malignant glomus tumor. She had an uneventful postoperative recovery. Options of treatment were reviewed by a multidisciplinary board. In light of no proven benefit for systemic therapy, she was referred for supportive care. She was under follow-up and she expired after 7 months due to progressive disease. Our literature review focuses on the clinicopathologic features and the current standard of management of malignant renal glomus tumors.</p>","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"11 1","pages":"33-40"},"PeriodicalIF":1.6,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10915652/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140050667","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 Narrative Review of Pheochromocytoma in VHL.","authors":"Danilo Coco, Silvana Leanza","doi":"10.15586/jkcvhl.v11i1.275","DOIUrl":"https://doi.org/10.15586/jkcvhl.v11i1.275","url":null,"abstract":"<p><p>This systematic review aims to investigate the clinical presentation, diagnostic methods, and management strategies for pheochromocytoma in patients with von Hippel-Lindau (VHL) disease, an autosomal dominant disorder that predisposes individuals to the development of various tumors, including pheochromocytomas. Pheochromocytoma is a rare neuroendocrine tumor of the adrenal medulla that occurs sporadically or as part of an inherited syndrome. The incidence of pheochromocytoma in VHL patients is estimated to be between 10-20%, making it the second most common tumor associated with VHL. Early detection and management of pheochromocytoma in VHL patients are critical for patient outcomes, as these tumors can cause severe hypertension, cardiovascular complications, and death. This review highlights the importance of screening for pheochromocytoma in VHL patients and discusses the current diagnostic and management strategies to optimize patient care.</p>","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"11 1","pages":"19-23"},"PeriodicalIF":1.6,"publicationDate":"2024-02-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10858801/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139724389","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}