Malignant Renal Tumors Research Articles

Outcomes of Pediatric Renal Tumors over 5 Years in Regional Cancer Center, Kidwai Memorial Institute of Oncology, Bengaluru, South India

Wilms' tumor (WT) is the most common malignant renal tumor in the pediatric population and it is managed with a multimodal treatment. Improvements in chemotherapy and risk stratification have led to dramatic improvement in the prognosis of renal tumors, which was once a lethal malignancy. Seventy-three patients with histopathologically proven diagnosis of renal tumor who received treatment from January 2011 to December 2015 were included for analysis. Eight children underwent upfront nephrectomy. The patients were analyzed for event-free survival and overall survival. The outcomes were correlated with age, sex, stage at presentation, and histology. A favorable histology was found in 74% patients, while an unfavorable histology was observed in 26% of the cases. The 5-year event-free survival was 82.7% and overall survival was 87.6%. The stage at presentation had a prognostic value (p < 0.001). Tumor histology was the single most important factor in predicting the survival.

A Case Report of Congenital Wilms’ Tumor and a Comprehensive Literature Review highlighting the spectrum between Wilms’ Tumor and Nephroblastomata’s

Abstract Introduction/Objective Wilms’ Tumour (WT) represents the most frequently encountered malignant renal neoplasm within pediatric pathology. To delineate the histopathological features of WT with perilobar nephrogenic rests (PLNR) and intralobar nephrogenic rests (ILNR) from nephroblastomatosis, we report a case with literature review to evaluate the clinical implications of WT with or without nephrogenic rests (NR), and nephroblastomatosis. Methods/Case Report Our patient is a 2-week-old neonate with VACTERL, germline mosaic triple X, and stage 1 WT with PLNR and substantial ILNR. The management approach involved radical nephrectomy exclusively. Obtaining a unifying clinical syndrome as well as deciding between WT or nephroblastomatosis was challenging in this case. The molecular testing with VACTERL panel was indeterminate and WT panel revealed DIS3L2 mutation that is indeterminate but likely pathogenic. She has been stable for 12 months and placed on 4-monthly left kidney ultrasound surveillance. Results (if a Case Study enter NA) Our literature review showed that the most common histologic subtype in WT patients without NR (n=59) and with NR (n=33) were classic triphasic (28.8%) and stromal-predominant (39.3%), respectively. WT patients with or without NR, underwent radical nephrectomy followed by adjuvant chemotherapy. Loss of heterozygosity (LOH) in TRIM28 was 4-fold higher in cases with NR. Mortality and relapse rates (12.5%) were higher in non-NR cohort. Conversely, nephroblastomatosis patients (n=14) underwent neoadjuvant chemotherapy, ipsilateral radical nephrectomy, and contralateral nephron-sparing surgery. Nephroblastomatosis patients had higher mortality (22.2%) and relapse rate (30%), with no identifiable mutations on molecular testing. Conclusion WT patients with NR have an earlier diagnosis and lower mortality and relapse rates compared to those without NR. The higher frequency of LOH in TRIM28 in WT patients with NR warrants future exploration to assess its implication in progression free survival. Nephroblastomatosis exhibits higher mortality and relapse rates, emphasizing the importance of reporting nephroblastomatosis in co-existing WT cases.

Spontaneous rupture and hemorrhage of renal epithelioid angiomyolipoma misdiagnosed to renal carcinoma: a case report

BackgroundRenal epithelioid angiomyolipoma is a rare and unique subtype of classic angiomyolipoma, characterized by the presence of epithelioid cells. It often presents with nonspecific symptoms and can be easily misdiagnosed due to its similarity to renal cell carcinoma and classic angiomyolipoma in clinical and radiological features. This case report is significant for its demonstration of the challenges in diagnosing epithelioid angiomyolipoma and its emphasis on the importance of accurate differentiation from renal cell carcinoma and classic angiomyolipoma.Case presentationA 58-year-old Asian female presented with sudden left flank pain and was initially diagnosed with a malignant renal tumor based on imaging studies. She underwent laparoscopic radical nephrectomy, and postoperative histopathology confirmed the diagnosis of epithelioid angiomyolipoma. The patient recovered well and is currently in good health with regular follow-ups. This case highlights the diagnostic challenges, with a focus on the clinical, radiological, and histopathological features that eventually led to the identification of epithelioid angiomyolipoma.ConclusionsEpithelioid angiomyolipoma is easily misdiagnosed in clinical work. When dealing with these patients, it is necessary to make a comprehensive diagnosis based on clinical symptoms, imaging manifestations, and pathological characteristics.

Frequency of benign tumors after partial nephrectomy and the association between malignant tumor findings and preoperative clinical parameters

BackgroundPartial nephrectomy (PN) has become the dominant treatment modality for cT1 renal tumor lesions. Tumors suspected of malignant potential are indicated for surgery, but some are histologically classified as benign lesions after surgery. This study aims to analyze the number of benign findings after PN according to definitive histology and to evaluate whether there is an association between malignant tumor findings and individual factors.MethodsThe retrospective study included 555 patients who underwent open or robotic-assisted PN for a tumor in our clinic from January 2013 to December 2020. The cohort was divided into groups according to definitive tumor histology (malignant tumors vs. benign lesions). The association of factors (age, sex, tumor size, R.E.N.A.L.) with the malignant potential of the tumor was further evaluated.ResultsIn total, 462 tumors were malignant (83%) and 93 benign (17%). Of the malignant tumors, 66% were clear-cell RCC (renal cell carcinoma), 12% papillary RCC, and 6% chromophobe RCC. The most common benign tumor was oncocytoma in 10% of patients, angiomyolipoma in 2%, and papillary adenoma in 1%. In univariate analysis, there was a higher risk of malignant tumor in males (OR 2.13, 95% CI 1.36–3.36, p = 0.001), a higher risk of malignancy in tumors larger than 20 mm (OR 2.32, 95% CI 1.43–3.74, p < 0.001), and a higher risk of malignancy in tumors evaluated by R.E.N.A.L. as tumors of intermediate or high complexity (OR 2.8, 95% CI 1.76–4.47, p < 0.001). In contrast, there was no association between older age and the risk of malignant renal tumor (p = 0.878).ConclusionsIn this group, 17% of tumors had benign histology. Male sex, tumor size greater than 20 mm, and intermediate or high R.E.N.A.L. complexity were statistically significant predictors of malignant tumor findings.

Value of elastography in characterization of solid renal masses

BackgroundSolid renal masses often come to light as incidental findings during abdominal ultrasound examinations. Once detected, determining whether a renal mass is benign or malignant becomes imperative for informed decision-making regarding management and treatment strategies. In this investigation, the aim was to explore the diagnostic efficacy of real-time strain sonoelastography in assessing solid renal masses.MethodsThis prospective research was steered on 26 individuals diagnosed with a solid renal mass, as endorsed by pathological analysis after surgical removal or biopsy. Elastography was performed on all patients. The measurement of strain index values for tissues was achieved by placing regions of interest of equal or near-equal size on both the tumor (A) and the adjacent normal renal cortex (B).ResultsStrain elastography showed no correlation with patient’s age, size of mass and probe to mass distance with p > 0.05 all. Sensitivity analysis showed that strain index can significantly predict malignant renal masses (P = 0.003) using a cut-off point 2, with 92.9% area under curve, 95.2% sensitivity, 80% specificity, 80% negative predictive value, 95.2% positive predictive value, and overall diagnostic accuracy 92.3%. Strain index > 2 was an independent predictor for malignant renal masses (P = 0.025), odds ratio 7.29 when adjusting for other risk factors. Malignant renal masses were significantly higher strain index compared to benign lesions with (P = 0.001).ConclusionsStrain elastography is a valuable technique for distinguishing between malignant and benign solid renal tumors. Benign lesions have lower strain index values compared to malignant ones, making the strain index a useful screening tool for distinguishing between benign and malignant renal masses using cut-off point 2.

The Clinicopathological Characteristics and Prognosis of 55 Patients With TFE3-Rearranged Renal Cell Carcinomas

ObjectiveTo explore the clinicopathological features and prognosis of TFE3-rearranged renal cell carcinomas (TFE3-rRCC). MethodsIn this retrospective observational study, the data of patients with TFE3-rRCC admitted to Xijing Hospital from January 2010 to October 2023 were collected, encompassing the general information, pathological diagnosis, immunohistochemistry, and the results of FISH detection. The treatment information and survival data of the patients were recorded during the follow-up. ResultsA total of 55 patients with TFE3-rRCC were enrolled, among whom 25 were males and 30 were females. TFE3 FISH assay suggested the disruption of the TFE3 gene. Fifty-four patients underwent surgical resection of kidney lesions, while 1 patient did not. By the end of follow-up in December 2023, 3 patients were lost to follow-up, 28 patients remained alive, and 24 patients had died. Among the 52 patients followed up, 31 developed metastases, involving lymph nodes, liver, bone, lung, peritoneum, pleura, adrenal gland, and brain. The 1-year and 5-year survival rates of the patients were 84.6% and 50.6%, respectively. In this study, there were 31 patients with TFE3-rRCC recurrence or metastasis. Median PFS was 7 and 13 months in the VEGFR-TKI and VEGFR-TKI+ ICI groups, respectively. The median OS was 12 months in the VEGFR-TKI treatment group. The median OS data of VEGFR-TKI+ ICI group has not been reached. The ORR and DCR was 25%, 66.7% in the VEGFR-TKI group. The ORR and DCR was 33.3%, 77.8% in the VEGFR-TKI+ ICI group. ConclusionTFE3-rRCC is a rare subtype of malignant renal tumor. The diagnosis mainly relies on pathological morphology, immunohistochemistry, and the detection of TFE3 gene disruption by FISH. In terms of treatment, surgery is the primary approach, and lymph nodes, liver, and bone are the main metastatic sites. VEGFR-TKI+ICI treatment might be an option of recurrent or metastatic TFE3-rRCC.

Renal bleeding: imaging and interventions in patients with tumors.

In patients with cancer, spontaneous renal bleeding can stem from a range of underlying factors, necessitating precise diagnostic tools for effective patient management. Benign and malignant renal tumors are among the primary culprits, with angiomyolipomas and renal cell carcinomas being the most common among them. Vascular anomalies, infections, ureteral obstructions, and coagulation disorders can also contribute to renal-related bleeding. Cross-sectional imaging techniques, particularly ultrasound and computed tomography (CT), play pivotal roles in the initial detection of renal bleeding. Magnetic resonance imaging and CT are preferred for follow-up evaluations and aid in detecting underlying enhancing masses. IV contrast-enhanced ultrasound can provide additional information for active bleeding detection and differentiation. This review article explores specific disorders associated with or resembling spontaneous acute renal bleeding in patients with renal tumors; it focuses on the significance of advanced imaging techniques in accurately identifying and characterizing renal bleeding in these individuals. It also provides insights into the clinical presentations, imaging findings, and treatment options for various causes of renal bleeding, aiming to enhance the understanding, diagnosis, and management of the issue.

Renal artery embolization in a case of renal cell carcinoma with intractable hematuria with acute myocardial infarction

Introduction: Renal artery embolization is a valuable treatment option for various renal tumors, both malignant and benign. The indications for this procedure include preoperative arterial embolization of large and vascular renal cell carcinoma to reduce intraoperative hemorrhagic complications, management of malignant renal tumors in patients who are not suitable candidates for surgery, treatment of symptomatic hematuria and palliative care for metastatic renal cancer. Case Report: In this case report, we present the case of a 56-year-old female with a known history of hypertension and cerebrovascular accident, who presented with complaints of hematuria and urinary retention. A diagnosis of left renal mass suggestive of renal cell carcinoma was made. The patient was planned for left radical nephrectomy and advised to discontinue aspirin for a week. However, on the morning of surgery, she experienced acute myocardial infarction and had to be hospitalized in intensive cardiac care. Aspirin was restarted, leading to recurrent severe hematuria. The patient underwent renal artery embolization, which successfully controlled the hematuria. Following cardiac stabilization, radical nephrectomy was performed three months later. The surgery and post-operative period were uneventful. Conclusion: This case study demonstrates the use of renal artery embolization as an effective modality for controlling hematuria, and temporizing management in a case of renal cell carcinoma where surgery was not immediately feasible due to a medical emergency.

Factors Associated with Outcomes at 1 Year in Paediatric Post-nephrectomy Patients for Nephroblastoma at the University Teaching Hospital and Cancer Diseases Hospital in Lusaka, Zambia.

Nephroblastoma is the most common primary malignant renal tumour of childhood. The survival rates in high-income countries are approximately 90%. However, low-income countries have low survival rates of 20%-50%. This study assessed factors associated with treatment outcomes of children post-nephrectomy for nephroblastoma at the University Teaching Hospital and Cancer Diseases Hospital in Lusaka, Zambia. A retrospective observational cohort study was conducted, where all children diagnosed with unilateral Wilms tumour below the age of 16 years who had nephrectomy from July 2016 to June 2019 were enrolled. Sociodemographic, clinical characteristics and treatment outcomes were noted. All data were coded and stored in a tabular format using Microsoft Excel. Statistical software STATA version 13 was used for analysis. Thirty patients were enrolled. The male-to-female ratio was 1:1. The 1-year event-free survival was 46.7%. Treatment abandonment accounted for 36.6% of the participants. 16.7% of the patients had disease progression. No patient had a relapse or died during the 1-year follow-up period. 66.7% had advanced disease stages III and IV. Advancement in age (above 4.3 years), living in a rural environment more than 100 km away from Lusaka and advanced disease stage were all associated with a poor outcome. Factors associated with a poor outcome in this study were advanced age and late presentation.

Tumoral EIF4EBP1 regulates the crosstalk between tumor-associated macrophages and tumor cells in MRTK

Malignant renal rhabdoid tumor (MRTK) is an aggressive and rare malignancy primarily affecting infants and young children. The intricate interactions within the Tumor Microenvironment (TME) are crucial in shaping MRTK's progression. This study elucidates the significance of tumor-associated macrophages(TAMs) within this milieu and their interplay with eukaryotic translation initiation factor 4E-binding protein 1 (EIF4EBP1) in tumor cells, collectively contributing to MRTK's malignant advancement. Through comprehensive analysis of clinical samples and the TARGET database, EIF4EBP1 emerges as a central macrophage-associated gene with robust prognostic implications. Elevated EIF4EBP1 expression correlates with poor prognosis and heightened infiltration of TAMs. Functional validation demonstrates that EIF4EBP1 knockdown in G401 cells significantly attenuates self-proliferation, migration, and invasion. Moreover, EIF4EBP1 regulates macrophage recruitment and M2 polarization through the ERK/P38 MAPK-MIF axis. Notably, M2 macrophages reciprocally foster the malignant behavior of MRTK tumor cells. This study unveils the pivotal role of EIF4EBP1 in propelling MRTK's malignant progression, unraveling a complex regulatory network involving EIF4EBP1 and TAMs. These findings underscore EIF4EBP1 as a promising biomarker and highlight its therapeutic potential in MRTK management.

Subsequent Renal Cancer Among Childhood Cancer Survivors: Analysis of Surveillance, Epidemiology, and End Results.

Renal cancer, although still rare among individuals under 45 years of age, is on the rise in the general population. The risk and timing of subsequent renal cancer in survivors of childhood cancer is not well established. Using the SEER registry, we reported the incidence of subsequent malignant renal neoplasms after treatment for primary malignancy diagnosed under 20 years of age. We evaluated clinical characteristics, standardized incidence ratio (SIR), and Kaplan-Meier survival estimates. Fifty-three survivors developed subsequent renal cancer (54 total cases). Of these, 54.7% were female, 88.7% were white, and 13.2% were Hispanic. Mean ages at primary malignancy and subsequent renal cancer were 10.1 and 31.1 years, respectively. Forty-seven cases were second cancers, 6 were third, and 1 was fourth. For survivors of childhood cancer, the overall SIR for renal cancer was 4.52 (95% CI: 3.39-5.89). The 5-year overall survival rate after development of subsequent renal cancer was 73% (95% CI: 58%-83%). Renal cancer occurs 4.5 times more frequently in childhood cancer survivors than in the general population, necessitating long-term care considerations.

Non-Wilms renal tumors: Twenty years experience in a referral center

BackgroundNon-Wilms Renal Tumors (NWRT) constitute less than 10% of all renal tumors diagnosed in childhood. We studied our experience in the diagnosis and management of these tumors to compare our results with the current literature. Study designThis is a retrospective observational study which includes all patients aged 0-18 years with histopathological diagnosis of NWRT treated in our center during the period 2000-2022. ResultsWe identified 10 patients with diagnosis of: cystic nephroma(3), congenital mesoblastic nephroma(1), renal cell carcinoma(2), clear cell sarcoma of the kidney (2), renal Ewing's sarcoma(1) and malignant rhabdoid tumor of the kidney(1). Sixty percent (60%) were female. The median age at diagnosis was 3.25 years (IQR 1.5-10). Median age at diagnosis excluding CN was 6 years old (IQR 2.62-10.75). Molecular alterations were detected in 60% of the cases. Hematuria (40%) and palpable abdominal mass (40%) were the most frequent presenting symptoms. In total, 62.5% patients were misdiagnosed during the preoperative period as Wilms' tumor (WT), based on imaging data. A Radical nephroureterectomy was performed in all cases and staging lymphadenectomy in 70%. We recorded a major complication in one patient, who suffered a contralateral lower renal pole infarction due to section of a polar vessel during surgery. This patient had no preoperative vascular study. The recurrence-free survival rate was 90% with a median follow-up of 6.4 years (IQR 2-13.9). DiscussionRadiological imaging has fundamental importance in the diagnosis of renal tumors, especially to identify children who might benefit from initial surgical treatment or the indication of biopsy for preoperative histopathological confirmation before initiating cytotoxic treatment. However, there are no pathognomonic imaging findings that clearly differentiate between WT and other renal tumors, nor among the heterogeneous group of NWRT[3]. In our series, 62.5% of patients were misdiagnosed as WT based on imaging features.We found different molecular alterations in 60% of our patients (Table 1). None of them predispose to the development of bilateral tumors nor correlates with predisposing syndromes of metachronous tumors.Apart from its retrospective design, this study is limited by small number of cases and a long study period. ConclusionsCorrect differential diagnosis of NWRT is necessary for an adequate therapeutic approach. The molecular-genetic profile is an important step in the diagnosis of NWRT that allows for the use of targeted therapies in refractory patients. Detailed anatomical study by vascular mapping minimizes the risk of iatrogenic damage during tumor resection.

Laparoscopic nephron-sparing surgery for complex renal cystic lesions: a single-center experience.

This study aimed to explore the feasibility and safety of laparoscopic nephron-sparing surgery (LNSS) for complex renal cystic lesions. A retrospective study was conducted on 83 cases of complex renal cystic lesions treated with LNSS in our hospital. There were 32 men and 51 women, ranging in age from 24 to 73years (average, 47.22±9.03years). The diameter of the cysts was 1.5-5.9cm (average, 3.44±0.86cm). According to the Bosniak classification, there were 15 cases of type II, 23 cases of type IIF, 29 cases of type III, and 16 cases of type IV complex renal cystic lesions. According to clinical classification based on the difficulty of laparoscopic partial nephrectomy and the depth of the lesion, the 83 complex renal cystic lesions were divided into 48 cases of the extra-renal type, 15 cases of the centrally located type, seven cases of the renal sinus type, and 13 cases of the renal hilum type. Laparoscopic partial nephrectomy was successful in all 83 patients. The surgical time was 35-102min (average, 52.13±14.38min), the intraoperative bleeding volume was 10-200ml (average, 27.25±12.26ml), and the renal artery occlusion time was 12-28min (average, 12.46±4.45min). There was no significant change in creatinine before and after surgery. The postoperative pathological results showed 71 cases of renal clear cell carcinoma, five cases of low malignant potential multilocular cystic renal tumors, and seven cases of pure renal cysts with all margins negative. There is potential for the malignant transformation of complex renal cysts into renal cell carcinoma. For complex renal cysts classified as Bosniak IIF or higher, surgical intervention is recommended, and LNSS is safe and effective. The complexity of the surgical procedure varies depending on the location classification of the complex renal cysts.

Interpretable multiphasic CT-based radiomic analysis for preoperatively differentiating benign and malignant solid renal tumors: a multicenter study

BackgroundTo develop and compare machine learning models based on triphasic contrast-enhanced CT (CECT) for distinguishing between benign and malignant renal tumors.Materials and MethodsIn total, 427 patients were enrolled from two medical centers: Center 1 (serving as the training set) and Center 2 (serving as the external validation set). First, 1781 radiomic features were individually extracted from corticomedullary phase (CP), nephrographic phase (NP), and excretory phase (EP) CECT images, after which 10 features were selected by the minimum redundancy maximum relevance method. Second, random forest (RF) models were constructed from single-phase features (CP, NP, and EP) as well as from the combination of features from all three phases (TP). Third, the RF models were assessed in the training and external validation sets. Finally, the internal prediction mechanisms of the models were explained by the SHapley Additive exPlanations (SHAP) approach.ResultsA total of 266 patients with renal tumors from Center 1 and 161 patients from Center 2 were included. In the training set, the AUCs of the RF models constructed from the CP, NP, EP, and TP features were 0.886, 0.912, 0.930, and 0.944, respectively. In the external validation set, the models achieved AUCs of 0.860, 0.821, 0.921, and 0.908, respectively. The “original_shape_Flatness” feature played the most important role in the prediction outcome for the RF model based on EP features according to the SHAP method.ConclusionsThe four RF models efficiently differentiated benign from malignant solid renal tumors, with the EP feature-based RF model displaying the best performance.Graphical

Clinical diagnostic value of contrast-enhanced ultrasound combined with microflow imaging in benign and malignant renal tumors: A retrospective cohort study.

This study aims to evaluate the clinical diagnostic value of contrast-enhanced ultrasound combined with microflow imaging (CEUS-MFI) in the differential diagnosis of benign and malignant renal tumors. All patients underwent CEUS, MFI, color doppler flow imaging (CDFI), and CEUS-MFI. The efficacies of these different diagnostic modalities in diagnosing benign and malignant renal tumors were evaluated by Kappa consistency test and the receiver operating characteristic (ROC) curve, with pathological findings serving as the gold standard. CDFI, MFI and CEUS-MFI all demonstrated higher blood flow in malignant tumors compared with benign tumors. Compared with benign tumors, CDFI detected a higher rate of punctate and linear Adler grade 2 and 3 blood flows in malignant tumors, as well as peripheral semicircular or annular blood flow. MFI identified a high rate of peripheral circumferential blood flow and irregular vascular morphology in malignant tumors, with most exhibiting Adler grade 3 blood flow. In addition, CEUS-MFI showed more dendritic or irregular Adler grade 2 or 3 blood flows in malignant renal tumors than MFI alone. Further analysis showed that CEUS-MFI had the highest consistency with pathological diagnosis (Kappa = 0.808). The ROC curve showed that the area under the curve (AUC) for CEUS-MFI in differentiating between benign and malignant lesions was 0.898, significantly outperforming other single diagnostic methods. With its capability to display microvascular information and assess overall pathological characteristics, MFI can accurately predict the nature of renal tumors and assist in surgical planning.

Clinical diagnostic value of contrast-enhanced ultrasound combined with microflow imaging in benign and malignant renal tumors: A retrospective cohort study.

This study aims to evaluate the clinical diagnostic value of contrast-enhanced ultrasound combined with microflow imaging (CEUS-MFI) in the differential diagnosis of benign and malignant renal tumors. All patients underwent CEUS, MFI, color doppler flow imaging (CDFI), and CEUS-MFI. The efficacies of these different diagnostic modalities in diagnosing benign and malignant renal tumors were evaluated by Kappa consistency test and the receiver operating characteristic (ROC) curve, with pathological findings serving as the gold standard. CDFI, MFI and CEUS-MFI all demonstrated higher blood flow in malignant tumors compared with benign tumors. Compared with benign tumors, CDFI detected a higher rate of punctate and linear Adler grade 2 and 3 blood flows in malignant tumors, as well as peripheral semicircular or annular blood flow. MFI identified a high rate of peripheral circumferential blood flow and irregular vascular morphology in malignant tumors, with most exhibiting Adler grade 3 blood flow. In addition, CEUS-MFI showed more dendritic or irregular Adler grade 2 or 3 blood flows in malignant renal tumors than MFI alone. Further analysis showed that CEUS-MFI had the highest consistency with pathological diagnosis (Kappa = 0.808). The ROC curve showed that the area under the curve (AUC) for CEUS-MFI in differentiating between benign and malignant lesions was 0.898, significantly outperforming other single diagnostic methods. With its capability to display microvascular information and assess overall pathological characteristics, MFI can accurately predict the nature of renal tumors and assist in surgical planning.

Prenatal Diagnosis of Renal Rhabdoid Tumor: A Rare Malignant Neoplasm

AbstractRhabdoid tumors of the kidney are highly lethal malignancies of infancy. We report the prenatal detection of this renal tumor in a fetus in the third trimester of pregnancy. Ultrasonologically, the tumor appeared as a large mass in the left renal area associated with severe polyhydramnios. Though the sonographic features alone did not allow distinction from a benign lesion, tumor extension into the subcutaneous plane favored the possibility of a malignant renal tumor and this was confirmed postnatally on histopathology.

Development and validation of a clinical and ultrasound features-based nomogram for preoperative differentiation of renal urothelial carcinoma and central renal cell carcinoma.

This study aimed to develop and validate an ultrasound (US)-based nomogram for the preoperative differentiation of renal urothelial carcinoma (rUC) from central renal cell carcinoma (c-RCC). Clinical data and US images of 655 patients with 655 histologically confirmed malignant renal tumors (521 c-RCCs and 134 rUCs) were collected and divided into training (n = 455) and validation (n = 200) cohorts according to examination dates. Conventional US and contrast-enhanced US (CEUS) tumor features were analyzed to determine those that could discriminate rUC from c-RCC. Least absolute shrinkage and selection operator regression was applied to screen clinical and US features for the differentiation of rUC from c-RCC. Using multivariate logistic regression analysis, a diagnostic model of rUC was constructed and visualized as a nomogram. The diagnostic model's performance was assessed in the training and validation cohorts by calculating the area under the receiver operating characteristic curve (AUC) and calibration plot. Decision curve analysis (DCA) was used to assess the clinical usefulness of the US-based nomogram. Seven features of both clinical features and ultrasound imaging were selected to build the diagnostic model. The nomogram achieved favorable discrimination in the training (AUC = 0.996, 95% CI: 0.993-0.999) and validation (AUC = 0.995, 95% CI: 0.974, 1.000) cohorts, and good calibration (Brier scores: 0.019 and 0.016, respectively). DCA demonstrated the clinical usefulness of the US-based nomogram. A noninvasive clinical and US-based nomogram combining conventional US and CEUS features possesses good predictive value for differentiating rUC from c-RCC.

Abstract 1738: Precise detection of benign and malignant renal tumor via epigenetic characteristics of urinary cell-free DNA

Abstract Early detection of clear cell renal cell carcinoma (ccRCC) and accurate differentiation between malignant and benign lesions pose clinical challenges. Profiling the epigenetic pattern of patients through liquid biopsy offers a promising strategy for non-invasive cancer identification. This study utilizes the methylation characteristics of urinary cell-free DNA to enhance the precise diagnosis of ccRCC. In this study, 32 patients with malignant RCC, 20 patients with benign kidney lesions, and 28 age- and sex-matched healthy donors were enrolled. Urine samples were collected and applied by PredicineEPIC assay to analyze the whole genome methylation profiles. We assessed the methylation differences on cancer-specific altered regions and constructed a machine learning model for malignant and benign classification. Beta values were calculated for differentially methylated regions identified through literature mining and our current work. These features carry methylation signals that are recognized by gradient boosting machine and used for classification training. The leave-one-out cross-validation method compensated for the shotage of sample size. The sensitivity and specificity of the optimal model in the training set are both greater than 0.97, and it can achieve an accuracy of 0.95 on the validation set. This performance surpasses traditional diagnostic methods such as contrast-enhanced CT and MRI. This study demonstrated the technical feasibility of leveraging epigenetics profiles to discriminate malignant from benign lesions, suggesting a promising liquid biopsy-based approach for the precise detection of renal cell carcinoma. Citation Format: Wen Kong, Hang Dong, Haoran Tang, Pan Du, Shidong Jia, Jin Zhang. Precise detection of benign and malignant renal tumor via epigenetic characteristics of urinary cell-free DNA [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2024; Part 1 (Regular Abstracts); 2024 Apr 5-10; San Diego, CA. Philadelphia (PA): AACR; Cancer Res 2024;84(6_Suppl):Abstract nr 1738.

Inoperable Renal Malignant Glomus Tumor, the answers for all the "W's"?

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.