搜索结果：Journal of kidney cancer and VHL

Early identification of patients at risk with von Hippel-Lindau (VHL) syndrome-related pheochromocytoma and paraganglioma (PPGL) is crucial to prevent morbidity. We investigated the current surveillance recommendations in VHL-related PPGL in children and adolescents. German Pediatric Oncology and Hematology-Malignant Endocrine Tumor registry (GPOH-MET) and Freiburg-VHL registry (1996-2022). In all, 75 patients (aged 0-18 years) with VHL syndrome were analyzed and 52 were in the Freiburg screening/surveillance program (median follow-up: 11.5 ± 0.94 years), including annual hormone level measurements, eye examination (starting at the age 6 years), and MRI of the abdomen and central nervous system (CNS) (starting at the age of 12 years). Retrospective analysis of clinical outcomes and descriptive statistics was performed. Of the 75 patients, 60 had a previous clinical diagnosis of PPGL with subsequent genetic testing, and 63% had a positive family history. In spite of having positive family history, large variations of timings between genetic and clinical diagnosis (range: -9 to +40 years) were observed. The mean age of first PPGL was 12.4 ± 0.41 years (range: 4-18 years). Recurrence of PPGL was common (46%; range: 2-7 per patient), and that of other tumors occurred: hemangioblastomas (73%), retinal angiomas (58%), renal cell carcinomas (21%), and pancreatic neuroendocrine tumors (12%). VHL-related PPGL appeared by the age of 12 and recurrences were observed frequently. Hemangioblastomas and retinal angiomas were common. In spite of a positive family history, VHL diagnoses were delayed. Because of high tumor proportions of affected families with children, it needs an optimization of the surveillance framework to enhance compliance and minimize anxiety and worse disease outcomes.

Von Hippel-Lindau (VHL) disease is a rare inherited syndrome characterized by benign and malignant neoplasms. Belzutifan, a HIF-2α inhibitor, was approved for the treatment of VHL-associated neoplasms. As a first-in-class agent, understanding tolerability and efficacy outside of a clinical trial setting and optimizing the management of adverse events (AEs) is important. We conducted a retrospective analysis of VHL patients ≥18 years old, treated with belzutifan at Vanderbilt University Medical Center, between November 2018 and December 2024. Clinical data and AEs were collected. Primary endpoint was safety; secondary endpoints included dose reduction, treatment interruption, treatment discontinuation, time to anemia onset, time to dose reduction, tumor shrinkage, objective response (per RECIST 1.1), and need for subsequent VHL-related procedures. Among 25 patients, with a median follow-up of 35.0 months, any-grade AEs occurred in 23 (92%) patients; anemia was the most frequent (64%, no grade ≥ 3). The median time to anemia onset was 3.7 months. Treatment interruption happened in 80% of the patients. The dose reduction was needed in 15 (60%) patients, with a median time of 6.8 months, and the median final dose was 80 mg. Tumor shrinkage occurred in 89% of RCC patients, 80% of CNS hemangioblastoma, and 80% of pancreatic neuroendocrine tumors (pNET). Overall, four (20%) patients experienced the progression of the disease. During follow-up, three (12%) patients required new VHL-related procedures. These findings support the long-term safety and efficacy of belzutifan in VHL disease, underscore the utility of dose reduction for AE management while demonstrating durable disease control, and a low incidence of interventional procedures.

Horseshoe kidney (HSK), characterized by the fusion of two kidneys forming a U-shape, presents intricate challenges in renal anatomy and poses a unique landscape for the development of renal cell carcinoma (RCC). This abstract delves into the case of a 74-year-old male with HSK who also developed RCC, where the employment of intraoperative retrograde pyelogram (RP) played a pivotal role in enhancing surgical precision. The patient's complex tumor was successfully resected through meticulous identification and dissection. A comprehensive literature review reveals the significance of laparoscopic and robotic surgeries in treating RCC within HSKs, with 3D-reconstruction aiding in surgical planning. While advancements in imaging technologies have improved surgical outcomes, the underexplored utility of intraoperative RP stands out. RP provided real-time insights into the renal pelvis anatomy, guiding the surgical team in navigating intricate structures and ensuring optimal reconstruction post-tumor excision. The discussion underscores the challenges posed by RCC in HSKs, importance of preoperative 3D-reconstruction and angiography in surgical planning, and the critical role of intraoperative RP in mapping renal pelvis anatomy. Unlike conventional imaging methods, RP offers dynamic visualization of the renal drainage system, safeguarding against inadvertent closures and enhancing surgical precision. The successful utilization of RP in this case not only facilitated safe tumor resection but also highlighted its potential in managing unclear renal structures. In conclusion, the integration of intraoperative RP in surgical interventions for RCC within HSKs proves instrumental in enhancing surgical precision and navigating complex anatomical variations. By emphasizing the importance of real-time imaging guidance, surgeons can optimize treatment outcomes for individuals with RCC in the challenging context of HSK anomalies.

For patients with advanced renal cell carcinoma, maintaining quality of life during treatment is a priority. The phase III CheckMate 9ER study demonstrated improved efficacy and health-related quality of life (HRQoL) outcomes with first-line cabozantinib plus nivolumab (CaboNivo) versus sunitinib (Sun) and underscored the need to improve understanding of the relationship between clinical efficacy and the patient treatment experience. In this work, we investigated the relationship between changes in HRQoL and clinical outcomes, as well as identifying symptom-based patient profiles that could predict disease course and clinical outcomes. This post hoc analysis of CheckMate 9ER used the patient-reported outcomes 19-item Functional Assessment of Cancer Therapy-Kidney Symptom Index (FKSI-19) to evaluate HRQoL. Cox proportional modeling was used to investigate the association between early changes in FKSI-19 scores and clinical outcomes, while latent class analysis (LCA) was used to identify symptom-based patient profiles. The nature of patient-reported symptoms was similar at baseline and 13 weeks for CaboNivo and Sun, but symptom frequency varied between arms. Early worsening of bone pain [hazard ratio (HR) 1.45, P = 0.010] and sleep quality (HR 1.45, P = 0.007) were associated with increased risk of mortality. The LCA identified three distinct subgroups of patients by symptom burden and risk profile. Patients in the limited symptoms class at week 13 were more likely to have received CaboNivo (limited: 63.4%; moderate-to-severe: 45.9%; severe: 38.5%), had a longer duration of treatment (>122 weeks: 37.2% versus 14.7% versus 17.9%), and were generally less likely to experience disease progression (61.6% versus 55.1% versus 76.9%) than other symptom classes. This post hoc analysis identified an association between efficacy and HRQoL based on data from CheckMate 9ER. These results highlight the necessity of balancing treatment choice, tolerability, and HRQoL to optimize treatment outcomes and support shared decision-making.

Robotic nephrectomy has become an increasingly preferred surgical technique for managing renal cell carcinoma (RCC). This review aims to systematically evaluate existing literature on the safety, efficacy, clinical outcomes, and associated costs of robotic nephrectomy, especially in relation to tumor dimensions and other pertinent patient factors. Following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, we performed an extensive literature search across major databases (PubMed, Scopus, and Cochrane Library) from inception to October 2023. The inclusion criteria encompassed randomized controlled trials (RCTs), cohort studies, and case-control studies that compared robotic nephrectomy with open or laparoscopic nephrectomy. Outcomes analyzed included operative time, intraoperative blood loss, complication rates, length of hospital stay, oncological outcomes, and cost-effectiveness. The Egger test was used to assess publication bias. The review incorporated 30 studies involving 5,432 patients who underwent robotic nephrectomy. Key findings indicated that robotic nephrectomy resulted in significantly reduced intraoperative blood loss (mean difference of -85 mL; p < 0.001) and shorter hospital stays (mean difference of -1.3 days). Tumor size had a notable impact on surgical outcomes, with larger tumors (&#x2265;7 cm) being associated with prolonged operative times and slightly higher complication rates. Robotic nephrectomy was also associated with higher costs compared to conventional surgical techniques; however, reduced readmission rates offset some of these costs. Oncological outcomes for robotic nephrectomy were comparable to those of open nephrectomy. Robotic nephrectomy is a safe and effective approach for kidney cancer that demonstrates advantages in perioperative recovery and surgical precision, particularly for smaller tumors. While costs may be higher, the clinical benefits and potential long-term savings from decreased postoperative complications recommend its use. Further high-quality RCTs are essential to validate these findings.

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) &#x2264; 25min or Cold ischemia time (CIT) &#x2264; 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.

Von Hippel-Lindau (vHL) is a hereditary disease characterized by the development of benign and malignant tumors across multiple organ systems. It is seen in approximately 1 in 36,000 live births. Given that vHL is a rare disease, studies that seek to characterize vHL are often hampered by small sample sizes. The TriNetX database, which contains data from over 100 million patients, may offer the ability to define and describe a large number of vHL patients. The primary objectives of this study were to describe the prevalence of vHL-associated conditions and investigate clinical outcomes using TriNetX. The secondary objective was to compare the results of this analysis to what has been reported in the published vHL literature. TriNetX was queried to establish a cohort of patients with a diagnosis of vHL. This cohort was then used to define the prevalence of the following conditions: reproductive organ (epididymal and broad ligament) cystadenomas, renal cell carcinoma (RCC), pheochromocytomas, endolymphatic sac tumors (ESLTs), central nervous system (CNS) and retinal hemangioblastomas, and pancreatic neuroendocrine tumor (pNETs). A total of 1232 patients in TriNetX had a recorded diagnosis of vHL. Of this, 34 (6.0% of males) patients had epididymal cystadenoma, 21 (3.4% of females) had broad ligament cystadenoma, 352 (28.6%) had RCC, 251 (20.4%) had pheochromocytoma, <10 had ELST, 171 (13.9%) had CNS hemangioblastoma, 34 (2.8%) had pNETs, and 66 (5.4%) had retinal hemangioma. Compared to the existing literature, vHL and associated conditions are underdiagnosed in TriNetX, suggesting its limited use in studying this disease.

The prevalence of papillary renal cell carcinomas (PRCCs) is estimated to be between 10% and 15%. At present, there is no effective therapeutic approach available for patients with advanced PRCCs. The molecular biomarkers associated with PRCC diagnoses have been rarely studied compared to renal clear cell carcinomas; therefore, the necessity for the identification of novel molecular biomarkers to aid in the early identification of this disease. Bioinformatics and artificial intelligence technologies have become increasingly important in the search for diagnostic biomarkers for early cancer detection. In this study, three genes-BCL11A, NTN5, and OGN-were identified as diagnostic biomarkers using the Cancer Genome Atlas (TCGA) database and deep learning techniques. To identify the differential expression genes (DEGs), ribonucleic acid (RNA) expression profiles of PRCC patients were analyzed using a machine learning approach. A number of molecular pathways and coexpressions of DEGs have been analyzed and a correlation between DEGs and clinical data has been determined. Diagnostic markers were then determined via machine learning analysis. The 10 genes selected with the highest variable importance value (more than 0.9) were further investigated, with six upregulated (BCL11A, NTN5, SEL1L3, SKA3, TAPBP, SEMA6A) and four downregulated (OGN, ADCY4, SMOC2, CCL23). A combined receiver operating characteristic (ROC) curve analysis revealed that the BCL11A-NTN5-OGN genes, which have specificity and sensitivity values of 0.968 and 0.901, respectively, can be used as a diagnostic biomarker for PRCC. In general, the genes introduced in this study may be used as diagnostic biomarkers for the early diagnosis of PRCC, thus providing the possibility of early treatment and preventing the progression of the disease.

To assess the surgical outcomes and techniques in managing renal cell carcinoma (RCC) with inferior vena cava (IVC) thrombus and bilateral renal tumors with a focus on the role of autotransplantation in complex cases, this retrospective study analyzed 58 patients treated at our center between 2013 and 2023 for RCC with tumor thrombus extending into the IVC and, in some cases, the right atrium (RA). Surgical management included radical nephrectomy and thrombectomy with techniques adapted to thrombus level. For level I and II thrombi, innovative occluding maneuvers were used to control the contralateral renal vein. For level IV thrombi, a beating heart technique combined with cardiopulmonary bypass (CPB) was employed. Of the 10 patients with bilateral renal tumors, 2 underwent autotransplantation and 8 underwent bilateral partial nephrectomy. In this 10-year retrospective study of 58 patients with either RCC with venous tumor extension or bilateral RCC, 40 males and 18 females, with a mean age of 66 &#xb1; 8 years. Tumor involvement was predominantly right-sided (72.4%). Thrombus levels included 53.44% Level I, 25.9% Level II, and 3.4% Level IV. Intraoperative and postoperative complications were minimal, affecting 10 patients; patients with Level I thrombus had a better survival rate; and one patient with Level IV thrombus died postoperatively. The mean blood loss was 360 mL and the mean operative time was 195 minutes. Histopathology revealed clear cell carcinoma in 65.5% of cases. Among the 10 patients with bilateral renal tumors, autotransplantation and partial nephrectomies resulted in excellent renal preservation and favorable outcomes. This study demonstrates the effectiveness of radical nephrectomy and thrombectomy for RCC with venous tumor extension. Tailored surgical techniques, including autotransplantation for bilateral tumors, resulted in excellent outcomes with minimal complications. Personalized surgical strategies were key to preserving renal function and improving survival in complex RCC cases.

The International Metastatic Renal Cell Carcinoma Database Consortium (IMDC) score is widely used for prognostic stratification in metastatic renal cell carcinoma (mRCC); however, the value of extended models incorporating metastatic site information remains uncertain in patients treated with first-line tyrosine kinase inhibitor (TKI) monotherapy. We retrospectively analyzed 183 patients with mRCC who received first-line TKI therapy (sunitinib, pazopanib, or cabozantinib) between 2013 and 2023. The overall survival (OS) and progression-free survival (PFS) were assessed using Kaplan-Meier analysis and Cox regression models, and the prognostic performance of IMDC, IMDC-7, and Meet-URO scores was compared using Harrell's concordance index. At baseline, 47% of patients had metastases to high-risk sites (bone, liver, or brain), and 36% had three or more metastatic sites. In univariate analyses, bone, liver, and brain metastases as well as the presence of &#x2265;3 metastatic sites were associated with significantly shorter OS and PFS. On multivariable analysis, anemia, poor performance status, absence of prior nephrectomy, and &#x2265;3 metastatic sites independently predicted worse OS, while anemia, poor performance status, and bone metastasis remained independently associated with inferior PFS. All three prognostic models effectively stratified survival outcomes; however, IMDC-7 demonstrated the highest discriminatory ability for OS, followed by IMDC and Meet-URO. These findings highlight the strong prognostic impact of both metastatic burden and distribution in mRCC and support the incorporation of metastatic site assessment into routine risk stratification, particularly in settings where access to immune-based combination therapies remains limited.

The von Hippel-Lindau (VHL) syndrome is a rare autosomal dominant disorder caused by mutations in the VHL tumor suppressor gene, leading to the development of benign and malignant tumors in multiple organs, including the kidneys, brain, spine, retina, and pancreas. Since its initial description in the early 20th century, significant progress has been made in understanding its pathogenesis, genetic basis, and clinical management. This narrative review provides a comprehensive overview of VHL syndrome, from its discovery to the latest medical and surgical therapies. A systematic literature review was conducted following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, incorporating the Egger test to assess publication bias. The review highlights the evolution of diagnostic criteria, the role of genetic testing, and the development of targeted therapies such as hypoxia-inducible factor 2-alpha (HIF-2&#x3b1;) inhibitors. Surgical interventions, including nephron-sparing surgery and minimally invasive techniques, are also discussed. This review emphasizes the importance of a multidisciplinary approach to managing VHL syndrome and explores emerging therapies that hold promise for improving patient outcomes.

Cutaneous leiomyomas (CLMs) are associated with Hereditary Leiomyomatosis and Renal Cell Cancer (HLRCC) syndrome (Mendelian Inheritance in Man [MIM]: 150800)-a rare genodermatosis caused by a heterozygous pathogenic variant in the fumarate hydratase (FH) gene. It is characterized by a predisposition to develop cutaneous and/or uterine leiomyomas and an aggressive type of renal cell carcinoma (RCC). We describe a 27-year-old male who presented with a painful nodule on the left upper arm persisting for 5 years and the subsequent emergence of painless nodules in various parts of the body over the past two years. A family history of RCC prompted suspicion of the HLRCC syndrome. Cutaneous examination revealed erythematous subcutaneous nodules, with histological analysis confirming CLM. Genetic testing identified a pathogenic variant in the FH gene, confirming the diagnosis of HLRCC. Management involved surgical excision of the symptomatic nodules and genetic counselling/testing for the proband and his family members. The long-term follow-up plan includes dermatological and nephrological surveillance with annual renal magnetic resonance imaging (MRI) scans. This report aims to enhance the awareness of this disease and highlight the role of cutaneous lesions in facilitating early detection.

Von Hippel-Lindau (VHL) disease is a hereditary condition caused by mutations in the VHL-tumor suppressor gene leading to constitutive overproduction of HIF-1alpha and HIF-2alpha, two proangiogenic factors, involved in the development of highly vascular tumors. Published evidence has shown that FSH-receptor is expressed in endothelial cells of blood vessels (BV) in several types of tumors. Given that VHL-associated tumors are highly vascular, it is plausible that FSH-receptor could be expressed in their vasculature as well. This immunohistochemical study involved 71 patients diagnosed with VHL-associated tumors, who required surgical intervention. Tissue specimens from these patients included CNS-hemangioblastoma, pancreatic neuroendocrine tumors (panNET), and clear cell renal cell carcinoma (ccRCC). Immunohistochemical staining was performed using a highly specific monoclonal antibody against the human FSH-receptor to assess its expression in the endothelial cells and tumor cells. The distribution of FSH-receptor staining was analyzed using digital imaging techniques. FSHR-protein expression was detected in the BV endothelial cells in 100% of VHL-associated CNS-hemangioblastoma, panNET, and ccRCC cases. In CNS-hemangioblastoma, 96% of cases showed FSH-receptor positivity in tumor stromal cells. In panNET, 88% of the cases displayed FSH-receptor expression in tumor cells. No tumor cells showed FSH-receptor expression in ccRCC. This is the first study to demonstrate FSH-receptor expression by cells of VHL-associated tumors, with distinct expression patterns in different tumor types.

Wilms' tumor is the most common renal malignancy in the pediatric age group. The unilateral type is the most prevalent, with a tenth of cases being a part of genetic malformation syndromes. The majority of Wilms' tumors are detected by chance detection of abdominal mass on ultrasound. Treatment protocols established by the Renal Tumor Committee of the Children's Oncology Group (COG) in North America and the International Society of Pediatric Oncology (SIOP) in Europe emphasize approaches such as surgical resection, neoadjuvant chemotherapy, and, in rare cases, irradiation. Radical nephrectomy with lymph node sampling forms the mainstay in the management of unilateral Wilms' tumor (uWT). Nephron-sparing surgery (NSS) is becoming increasingly popular for unilateral pediatric cases due to its ability to preserve functional renal tissue. This article reviews various recent studies that explored the role of NSS in uWT. Additionally, two case studies involving NSS for uWT and their outcomes are presented. This study found that factors such as the timing and duration of neoadjuvant chemotherapy, tumor mass size, presence of tumor margins, and intraoperative ischemia time significantly influenced the outcome of NSS.

Hypoxia creates a stressful environment for the cells triggering adaptive changes in the transcription factors called hypoxia inducible factors (HIF), which help to meet the metabolic and angiogenic requirements of cells. HIF-2 is one such factor that is implicated in the progression of various cancers, especially the ones associated with Von Hippel-Lindau (VHL) disease. HIF-2 factor has an unstable component alpha and a stable component beta. HIF-2&#x3b1; detects hypoxia and dimerizes with HIF-beta (Aryl hydrocarbon receptor nuclear translocator [ARNT]) through per-ARNT-sim (PAS)-mediated signaling domains. These domain sites are recognized as targets for HIF-2 inhibitors. HIF-2 inhibitors block this heterodimerization and prevent the expression of target genes which are oncogenic, including VEGF, PDGF, CAIX, and Oct4. VHL disease caused by the deficiency of VHL gene product results in decreased degradation of HIF-2&#x3b1;, leading to increased activation of these transcription factors. Tumors driven by the deficiency of VHL gene product are natural candidates for HIF-2 inhibitor therapy. These inhibitors have emerged as a promising class of targeted therapies for renal cell carcinoma (RCC), particularly in cases resistant to conventional treatments. In this review, we explore the role of hypoxia and HIF transcription factors in tumor formation and progression, highlighting the role and development of HIF-2 pathway inhibitors as potential cancer therapies. We discuss the major key inhibitors, with focus on belzutifan and review the various trials investigating its efficacy in monotherapy as well as in combination therapies in RCC. Additionally, we explore its development in pheochromocytoma, hemangioblastoma, and pancreatic neuroendocrine tumors. We also highlight emerging HIF-2&#x3b1; inhibitors currently in clinical trials, namely casdatifan, NKT-2152, and DFF332. Finally, we address the major toxicities and management of these inhibitors.

Renal cell carcinoma (RCC) in children is rare, comprising only 1.4-6.3% of pediatric renal tumors. Differentiating RCC from Wilms tumor, the most common pediatric renal tumor is crucial due to differing management and prognosis. Imaging characteristics, such as the presence of calcifications and cystic components, guided the decision to perform surgery without pretreatment biopsy, reducing the risk of needle tract seeding. Translocation-associated RCC is the most common subtype in children, and surgical resection remains the cornerstone of treatment. Long-term follow-up is essential due to the potential for late recurrences. We are reporting the details of a 4-year-old boy, who presented with a 15-day history of fever and abdominal distension, accompanied by a ballotable mass in the right lumbar region. Imaging studies, including a CT scan, revealed a large complex cystic mass in the right kidney, consistent with Bosniak category IV, and enlarged paraaortic nodes. Further evaluation with an FDG-PET scan confirmed the uptake only in the right kidney. The child underwent a right radical nephrectomy with retroperitoneal lymph node dissection. Histopathology revealed translocation-associated RCC features, characterized by slender papillae, psammoma bodies, necrosis, and uniform epithelial cells with hyperchromatic nuclei. Immunohistochemistry showed positivity for markers including TEF3, keratin, and vimentin, with a KI-67 proliferation index of 10-20%. The final stage was pT2aN0, and the patient had an uneventful recovery, with no recurrence at 36 months of follow-up.

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.

Enhancing renal masses are conventionally treated as malignant unless proven otherwise due to the difficulty distinguishing between malignant and benign tumors based on imaging. Data from the Western registries suggests overtreatment of renal tumors with a Benign Kidney Tumor Resection Rate (BKTRR) ranging from 10 to 33%, with an increasing trend. Since robust, population-based data from India was unavailable, we sought to determine BKTRR in an apex cancer institute, which would provide insight into the rates in the community. The institutional kidney tumor database was queried for all patients aged &#x2265;18 years with renal neoplasms between January 2000 and December 2022. Patients who underwent surgery, either radical or partial nephrectomy, with intent to cure were analyzed and the BKTRR during the study period was evaluated. A total of 330 patients underwent surgery for renal tumors presumed to be malignant. A final pathologic diagnosis of the benign tumor was made in 16 (4.8%) patients, comprising 7.2, 7.2, and 3.7% of resections with LTD &#x2264;4, 4-7, and >7 cm, respectively. Asymptomatic benign tumors &#x2264;7 cm comprised 3.0% of all resections, and these were potentially unnecessary surgeries. A multivariable analysis suggested that no patient or imaging characteristic could predict a final benign extirpative pathology. Our study suggests a lower rate of BKTRR compared to the published international literature but is likely to be the lower limit of that in the community. Population-based studies are required to determine the true BKTRR and the quantum of potentially unnecessary surgeries for benign kidney tumors.

Primary renal fibrosarcoma is an exceedingly rare malignant mesenchymal tumor that accounts for only 1-3% of adult renal malignancies, often mistaken for sarcomatoid renal cell carcinoma (RCC) or leiomyosarcoma due to overlapping morphology. Thus, accurate disease diagnosis is crucial for its management. We report a case of a 49-year-old female, a chronic smoker, who presented with right flank pain and progressive abdominal swelling. Clinical examination revealed a firm mass in the right abdomen, and contrast-enhanced computed tomography demonstrated a large exophytic right renal mass with areas of necrosis, infiltration of the psoas muscle, and extension of tumor thrombus into the inferior vena cava (IVC). The patient underwent right radical nephrectomy with IVC thrombectomy, and a 20 &#xd7; 15 cm irregular renal mass with 2 cm IVC thrombus was excised intraoperatively. Histopathology revealed a high-grade spindle cell neoplasm with interlacing fascicles and herringbone patterns, brisk mitoses (10-12 mitotic figures per 10HPF), and 20% necrosis. Immunohistochemistry showed diffuse vimentin positivity with negative staining for epithelial, myogenic, neural, and renal lineage markers, confirming the diagnosis of high-grade primary renal fibrosarcoma. This case is notable for being the largest renal fibrosarcoma reported to date, with rare IVC extension, features typically associated with advanced RCC rather than fibrosarcoma. Despite aggressive pathology, no metastases were identified, and the patient remained recurrence-free at 6 months postoperatively, with chemotherapy reserved for recurrence or metastasis. This report emphasizes the diagnostic challenges, surgical complexity, and clinical significance of primary renal fibrosarcoma and highlights the importance of including it in the differential diagnosis of large renal masses with vascular involvement.