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Purpose: To evaluate the association between the newly developed region of interest (ROI)-modified Mayo Adhesive Probability (MAP) score, in which stranding was re-evaluated by computed tomography (CT) number, for predicting operation time in robot-assisted partial nephrectomy (RAPN). Methods: The study participants were 119 patients who underwent transperitoneal RAPN. With regard to stranding, ROIs were evaluated, and the mean CT numbers were assigned a score ranging from 0 to 3. Clinical variables were evaluated in a multivariate logistic regression analysis in relation to prolonged operation time. Results: The percentage of patients with score ≥ 3 by MAP score alone was significantly higher than those of patients with score ≥ 3 by ROI-modified MAP score alone (26.8% vs. 13.4%, p < 0.001). Multivariate analysis revealed no independent association with the MAP score. On the other hand, for ROI-modified MAP score, score ≥ 3 was an independent factor for prolonged operation time (OR = 4.28, p = 0.0032) along with body mass index (BMI) ≥ 22 (OR = 4.46, p = 0.01), R.E.N.A.L. nephrometry score ≥ 7 (OR = 4.12, p = 0.0047), posterior tumor location (OR = 2.85, p = 0.036), and clinical T stage ≥ 1b (OR = 6.19, p = 0.0044). Regarding the predictive performance, the accuracy of the ROI-modified MAP score was significantly higher than the MAP score (area under the curve [AUC] value: 0.652 vs. 0.721, p = 0.034). Conclusion: The ROI-modified MAP score was a more relevant factor regarding operation time, suggesting that it might be a better preoperative predictor. [ABSTRACT FROM AUTHOR]

Background: The purpose of this article is to overview the clinical significance of left supraclavicular adenopathy and review the etiology of inferior vena cava (IVC) thrombosis, starting from a presentation of a rare case of renal cell carcinoma (RCCs) with Xp11.2 translocation involving TFE3 gene fusion. This article also aims to review the literature to understand the characteristics of this rare type of renal tumor. Renal cell carcinoma (RCC) associated with Xp11.2 translocation/gene fusion TFE3 is a rare subtype of kidney cancer that was classified in 2016 as belonging to the family of renal carcinomas with MiT gene translocation (microphthalmia-associated transcription factor). The prognosis for these kidney cancers is poorer compared to other types. Methods: We present a case of a 66-year-old man with Virchow–Troisier adenopathy during physical examination, which raises the suspicion of infra-diaphragmatic tumor. The echocardiography highlighted a heterogeneous mass in the right cardiac cavities, and the abdominal ultrasound exam revealed a solid mass at the upper pole of the left kidney. Results: Following computed tomography, magnetic resonance imaging, PET-CT, and histopathological and immunohistochemical examinations, the patient was diagnosed with renal carcinoma with Xp11.2 translocation and TFE3 gene fusion. Conclusions: IVC thrombosis is often associated with neoplastic disease due to the procoagulant state of these patients, the most common malignancies related to IVC thrombosis being represented by RCCs (38%), genitourinary cancers (25%), bronchus and lung cancers, retroperitoneal leiomyosarcoma, and adrenal cortical carcinoma. Imaging methods play a crucial role in differential diagnosis, allowing for the localization of the primary tumor and assessment of its characteristics. [ABSTRACT FROM AUTHOR]

Dysregulated specificity proteins (SPs), members of the C2H2 zinc-finger family, are crucial transcription factors (TFs) with implications for renal physiology and diseases. This comprehensive review focuses on the role of SP family members, particularly SP1 and SP3, in renal physiology and pathology. A detailed analysis of their expression and cellular localization in the healthy human kidney is presented, highlighting their involvement in fatty acid metabolism, electrolyte regulation, and the synthesis of important molecules. The review also delves into the diverse roles of SPs in various renal diseases, including renal ischemia/reperfusion injury, diabetic nephropathy, renal interstitial fibrosis, and lupus nephritis, elucidating their molecular mechanisms and potential as therapeutic targets. The review further discusses pharmacological modulation of SPs and its implications for treatment. Our findings provide a comprehensive understanding of SPs in renal health and disease, offering new avenues for targeted therapeutic interventions and precision medicine in nephrology. [ABSTRACT FROM AUTHOR]

We describe two cases in which surgery was performed to treat renal tumors wherein a pathological examination revealed the presence of different types of renal tumor within the same kidney. First case : A man in his 70’s visited his local doctor with hematuria and left back pain. He was referred to our department after various examinations revealed bilateral lesions suggestive of renal tumors. The left kidney was found to have one mass measuring 89 mm in size, and the right kidney had one tumor measuring 31 mm, extending over the lateral and dorsal sides of the center. Considering the remaining renal function, we decided to perform left laparoscopic partial nephrectomy and two-stage laparoscopic radical right nephrectomy. The pathology of the left kidney was clear cell renal cell carcinoma, while that of the right kidney was clear cell renal cell carcinoma on the outer center and papillary renal cell carcinoma on the dorsal side. Second case : A woman in her 50’s was suspected of having a right renal tumor after echocardiography and was referred to us. Contrast computed tomography and magnetic resonance imaging showed a 47-mm large mass lesion on the dorsal inferior pole of the right kidney and another 10-mm large mass lesion on the ventral side. A partial abdominal right nephrectomy was performed, and the pathology revealed a dorsal hemangiomyolipoma and renal cell carcinoma. This case is reported together with a review of the literature and the pathological features of the case. [ABSTRACT FROM AUTHOR]

Purpose: This study retrospectively assessed the diagnostic accuracy of fat quantification based on proton density fat fraction (PDFF) for differentiating renal tumors. Methods: In this retrospective study, 98 histologically confirmed clear cell renal cell carcinomas (ccRCCs), 35 papillary renal cell carcinomas (pRCCs), 14 renal oncocytomas, 16 chromophobe renal cell carcinomas (chRCCs), 10 lymphomas, 19 uroepithelial tumors, 10 lipid-poor angiomyolipomas (AMLs), and 25 lipid-rich AMLs were identified in 226 patients (127 males and 99 females) over 5 years. All patients underwent multiparametric kidney MRI. The MRI protocol included an axial plane and a volumetric 3D fat fraction sequence known as mDIXON-Quant for PDFF measurement. Demographic data were recorded, and PDFF values were independently reviewed by two radiologists blinded to pathologic results. MRI examinations were performed using a 1.5 T system. MRI-PDFF measurements were obtained from the solid parts of all renal tumors. Fat quantification was performed using a standard region of interest for each tumor, compared to histopathological diagnoses. Sensitivity and specificity analyses were performed to calculate the diagnostic accuracy for each histopathological tumor type. Nonparametric variables were compared among the subgroups using the Kruskal–Wallis H test and Mann Whitney U test. P-values < 0.05 were considered statistically significant. Results: In all, 102 patients underwent partial nephrectomy, 70 patients underwent radical nephrectomy, and the remaining 54 had biopsies. Patient age (mean: 58.11 years; range: 18–87 years) and tumor size (mean: 29.5 mm; range: 14–147 mm) did not significantly differ across groups. All measurements exhibited good interobserver agreement. The mean ccRCC MRI-PDFF was 12.6 ± 5.06% (range: 11.58–13.61%), the mean pRCC MRI-PDFF was 2.72 ± 2.42% (range: 2.12–3.32%), and the mean chRCC MRI-PDFF was 1.8 ± 1.4% (range: 1.09–2.5%). Clear cell RCCs presented a significantly higher fat ratio than other RCC types, uroepithelial tumors, lymphomas, and lipid-poor AMLs (p < 0.05). Lipid-rich AMLs demonstrated a very high fat ratio. Conclusion: MRI-PDFF facilitated accurate differentiation of ccRCCs from other renal tumors with high sensitivity and specificity. [ABSTRACT FROM AUTHOR]

Abstract Background Birt-Hogg-Dube (BHD) syndrome is a rare genetic condition associated with the development of renal tumors. This study aims to determine typical age ranges for detecting renal abnormalities, risk factors for tumor development, and long-term outcomes based on current surveillance strategies. Methods A single-center multi-site retrospective cohort study was performed on all patients with BHD diagnosed from 2000 to 2023. Baseline demographics, pulmonary function, laboratory, radiologic, and histopathologic findings were collected. Logistic regression was used to assess predictor variables for the development of renal tumors with survival analysis evaluated from the date of BHD diagnosis to date of death or last known follow-up. Results The study included 149 patients with BHD, 39 (26%) with diagnosed renal tumors, of which 28 had histopathologic confirmation. Mean age at renal tumor detection was 53.61 years. Older age and male sex were predictive of renal tumor development ((odds ratio 1.05; 95% CI, 1.01–1.08, P = 0.002) and (odds ratio 2.59; 95% CI, 1.17–5.73, P = 0.02), respectively). Time to all-cause mortality appeared shorter in those with renal tumors (Log-rank P = 0.02), though no deaths were from cancer or cancer-related complications. Conclusions Current screening protocols for renal tumors in BHD suggest the most common presenting age range for presentation is late 40s to early 50s, with older age and male sex as risk factors for tumor development.

Abstract Background Robot-assisted surgery (RAS) is being performed with increasing frequency in pediatric oncology. We report our experience with RAS for renal tumors in children and compare the outcomes between RAS and laparoscopic surgery (LAS). Methods A total of 23 patients with renal tumor who underwent minimally invasive surgery (MIS) between January 2020 and December 2023 were included in the study. The inclusion criteria enrolled in this study was unilateral tumors with maximum tumor diameter less than 10 cm. Patients who had enlarged lymph node, venous thrombosis, preoperative tumor rupture, bilateral renal tumor, or extrarenal extension on imaging were deemed contraindications and excluded. Patient demographics, operative details, postoperative outcomes and follow-up were recorded. Results Among these patients, 17 underwent RAS and 6 underwent LAS. In the RAS group, the median age was 64 months (range, 9–156) with a median weight of 19.48 kg (range, 8.4–46.5); the maximum tumor diameter at operation was 55.65 mm (range, 22–88); the operation time was 188.8 min (range, 120–210), the intraoperative blood loss was 20 ml (range, 5–50), and the length of postoperative hospital stay was 4 days (range 1–9). There was no significant difference in patients’ age, weight, location, tumor size, histological pattern and operation time between the two groups (P > 0.05). The RAS group had a significantly less intraoperative blood loss (P = 0.026) and less length of postoperative stay (P = 0.01) than the LAS group. Conclusion Our initial experience suggested that RAS in pediatric renal tumor was feasible and safe, and it reduced surgical trauma and accelerate postoperative recovery for the patients. Due to the limitations of sample size and study quality, the clinical importance of these findings still needs to be further verified.

S100A9 is an important alarmin in vivo, which plays a role in regulating inflammation and tumorigenesis. Recently, many studies have also explored the biological function and related mechanism of S100A9 in renal diseases, including acute kidney injury, chronic kidney disease, renal stone, renal transplantation, renal tumor, renal cyst, and urinary infection. They pointed out the potential role of S100A9 as a new diagnostic and therapeutic biomarker for renal diseases. In the current study, we conduct a review of these findings and summarize possible future study directions, which aims to help people understand the impact of S100A9 on renal diseases. [ABSTRACT FROM AUTHOR]

Context: Contrast-enhanced ultrasound (CEUS) is a cost-effective radiation-free diagnostic method that can be used for renal tumor postoperative visualization after ablative treatment. Objective: To assess CEUS diagnostic accuracy comparing with CT and MRI as a follow-up method in short-term and long-term postoperative periods after renal tumor ablation. Materials and methods: A systematic review and meta-analysis were performed in Scopus and Medline databases using the query "(kidney OR rena* OR RCC) AND (ablation OR RFA OR MWA OR cryo*) AND CEUS". The endpoint of the study was the evaluation of the overall accuracy of CEUS. Results: Twelve trials were included in the review. With CT or MRI as a reference, for a short-term group (< 6 weeks after ablation) pooled sensitivity was 90.2%, I2 = 0%; pooled specificity was 99.3%, I2 = 0%; pooled NPV was 98.6%, I2 = 0%; pooled PPV was 94.6%, I2 = 0%; the AUC on the SROC curve was 0.971. For the long-term group (> 6 weeks after ablation), pooled sensitivity was 95.3%, I2 = 0%; pooled specificity was 97.6%, I2 = 0%; PPV was 74.2%, I2 = 4%; NPV was 99.4%, I2 = 5%; AUC = 0.93. Conclusion: CEUS has high sensitivity and specificity in ruling out the presence of local recurrence after renal tumor ablation with a higher risk of false-positive results within follow-up > 6 weeks compared with that for CT or MRI. Further studies with a unified protocol and morphological control of local renal tumor recurrence after ablation are needed. [ABSTRACT FROM AUTHOR]

Background: Robot-assisted surgery (RAS) is being performed with increasing frequency in pediatric oncology. We report our experience with RAS for renal tumors in children and compare the outcomes between RAS and laparoscopic surgery (LAS). Methods: A total of 23 patients with renal tumor who underwent minimally invasive surgery (MIS) between January 2020 and December 2023 were included in the study. The inclusion criteria enrolled in this study was unilateral tumors with maximum tumor diameter less than 10 cm. Patients who had enlarged lymph node, venous thrombosis, preoperative tumor rupture, bilateral renal tumor, or extrarenal extension on imaging were deemed contraindications and excluded. Patient demographics, operative details, postoperative outcomes and follow-up were recorded. Results: Among these patients, 17 underwent RAS and 6 underwent LAS. In the RAS group, the median age was 64 months (range, 9–156) with a median weight of 19.48 kg (range, 8.4–46.5); the maximum tumor diameter at operation was 55.65 mm (range, 22–88); the operation time was 188.8 min (range, 120–210), the intraoperative blood loss was 20 ml (range, 5–50), and the length of postoperative hospital stay was 4 days (range 1–9). There was no significant difference in patients' age, weight, location, tumor size, histological pattern and operation time between the two groups (P > 0.05). The RAS group had a significantly less intraoperative blood loss (P = 0.026) and less length of postoperative stay (P = 0.01) than the LAS group. Conclusion: Our initial experience suggested that RAS in pediatric renal tumor was feasible and safe, and it reduced surgical trauma and accelerate postoperative recovery for the patients. Due to the limitations of sample size and study quality, the clinical importance of these findings still needs to be further verified. [ABSTRACT FROM AUTHOR]

Purpose: Indeterminate renal masses are increasingly incidentally found on cross-sectional imaging. 99mTc-sestamibi single-photon emission computed tomography/computed tomography (SPECT/CT) scans can be used to identify oncocytomas and oncocytic renal neoplasms, including a subset of chromophobe renal cell carcinomas (chRCCs), which are viewed as false-positive. Procedure: Patients imaged with renal sestamibi scans between 2014 and 2023 were reviewed. Those patients with solitary tumors that were originally classified as chRCC were included in the analysis. Imaging with SPECT/CT from the liver dome down had been carried out 75 min after the administration of 925 MBq of 99mTc-sestamibi. All available H&E and immunostained slides were re-reviewed and classified according to WHO 2022 criteria. Confirmatory immunohistochemical stains were performed in tumors considered morphologically suspicious for non-chRCC entities. Result: A total of 18 patients with solitary tumors were included in the final analysis. 13/18 (72.2%) tumors in this cohort remained classified as chRCC, with 4/18 (22.2%) being eosinophilic-variant chRCC. The reclassified tumors (5/18 [27.8%]) included 2/18 (11.1%) low-grade oncocytic tumor (LOT), 1/18 (5.5%) eosinophilic vacuolated tumor (EVT), and 2/18 (11.1%) unclassified low-grade oncocytic neoplasms. As such, only 2/9 (22.2%) qualitatively "hot" tumors were chRCC other than eosinophilic-variant and only 1/9 (11.1%) "cold" tumors was a histology other than chRCC. Conclusion: Based on current histopathologic classification methods, it is likely that the "false-positive" rate of uptake on renal sestamibi scans with chRCC has been over-stated. Further study is warranted to better refine the optimal utility of renal sestamibi scans for non-invasive risk stratification of indeterminate renal masses. [ABSTRACT FROM AUTHOR]

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 ≥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 ≤4, 4–7, and >7 cm, respectively. Asymptomatic benign tumors ≤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. [ABSTRACT FROM AUTHOR]

Background: Birt-Hogg-Dube (BHD) syndrome is a rare genetic condition associated with the development of renal tumors. This study aims to determine typical age ranges for detecting renal abnormalities, risk factors for tumor development, and long-term outcomes based on current surveillance strategies. Methods: A single-center multi-site retrospective cohort study was performed on all patients with BHD diagnosed from 2000 to 2023. Baseline demographics, pulmonary function, laboratory, radiologic, and histopathologic findings were collected. Logistic regression was used to assess predictor variables for the development of renal tumors with survival analysis evaluated from the date of BHD diagnosis to date of death or last known follow-up. Results: The study included 149 patients with BHD, 39 (26%) with diagnosed renal tumors, of which 28 had histopathologic confirmation. Mean age at renal tumor detection was 53.61 years. Older age and male sex were predictive of renal tumor development ((odds ratio 1.05; 95% CI, 1.01–1.08, P = 0.002) and (odds ratio 2.59; 95% CI, 1.17–5.73, P = 0.02), respectively). Time to all-cause mortality appeared shorter in those with renal tumors (Log-rank P = 0.02), though no deaths were from cancer or cancer-related complications. Conclusions: Current screening protocols for renal tumors in BHD suggest the most common presenting age range for presentation is late 40s to early 50s, with older age and male sex as risk factors for tumor development. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate retrospectively the influence of percutaneous cryoablation for small renal tumors on total and affected kidney function and risk factors associated with worsening function of the affected kidney. Materials and methods: Between April 2016 and March 2022, 27 patients who underwent cryoablation for small renal tumors at our institution participated in this study, which investigated time-dependent changes in postoperative renal function. We evaluated estimated glomerular filtration rates (eGFRs) and split renal function revealed by scintigraphy using 99 m technetium-mercaptoacetyltriglycine (99mTc-MAG3) before cryoablation and at 1 week, 1 month, and 6 months after cryoablation. Numerous variables were analyzed to assess risk factors for worsening renal function. Results: Baseline eGFR (mean ± standard deviation) was 56.5 ± 23.7 mL/min/1.73 m2 (mean ± SD; range, 20.5–112.5). Mean eGFRs at 1 week, 1 month, and 6 months after cryoablation were 57.4 ± 24.5 (19.1–114.9), 57.1 ± 25.1 (21.5–114.9), and 53.8 ± 23.9 mL/min/1.73 m2 (20.0–107.5), respectively. Changes were statistically insignificant (p = 1.0000, = 0.6749, and = 0.0761, respectively). Regarding split renal function, mean baseline contribution of the affected kidney determined by 99mTc-MAG3 was 49.7% ± 6.0% (38.8–63.3%); these rates at 1 week, 1 month, and 6 months after cryoablation were 43.7% ± 8.8 (29.1–70.6%), 46.2% ± 7.7% (32.6–70.3%), and 46.0% ± 8.5% (32.5–67.6%), respectively. Differences from baseline were significant for all periods (p < 0001, < 0001, = 0.0001, respectively). Serum C reactive protein and lactate dehydrogenase at 1 day following cryoablation, tumor's nearness to the collecting system or sinus, and volume of ablated normal renal parenchyma were significantly correlated with decreased contributions of the affected kidney by > 10% after cryoablation. Conclusion: Unlike total renal function, affected kidney function could worsen after cryoablation. [ABSTRACT FROM AUTHOR]

Renal angiomyolipoma (AML), also referred to as renal hamartoma, is a rare solid tumor without malignant characteristics. The inheritance pattern of renal AML is autosomal dominant. If the lesion grows to a large size, a series of clinical manifestations and serious complications may occur. We herein present a case of giant renal AML in a 49-year-old female patient, who presented with left-sided abdominal bloating for 1 month. Following abdominal ultrasound and computed tomography examination, the patient underwent total left nephrectomy. The resected mass was sized 18 × 15 × 11 cm. Postoperative histopathological examination confirmed the lesion as a giant renal AML. There are only few cases of giant renal AML (> 10 cm) reported in the literature to date. Due to the large size of the tumor, it is crucial to report similar cases, their diagnosis, and treatment. [ABSTRACT FROM AUTHOR]

Background: Giant abdominal tumors exceeding 10 cm in diameter are rare and often involve several critical organs and vascular structures. The primary treatment is surgical excision, which presents substantial challenges, including organ preservation, hemorrhage control, and intact tumor removal. Case summary: We present a 46-year-old male with a massive abdominal mass invading the right kidney and inferior vena cava (IVC). To address this, we developed a novel technique using continuous renal replacement therapy (CRRT) renal vein (RV) shunt procedure. A temporary dialysis catheter was placed in the internal jugular vein to divert blood from the left RV to the superior vena cava. After excising the mass and part of the IVC, an artificial blood vessel was used to reconnect the left RV to the IVC. The urine output of the patient was normal during the surgery, and the renal function remained normal during the 343 days' follow-up period. This approach streamlined the surgical procedures, operating time, blood loss, and avoided systemic heparinization through regional citrate anticoagulation. Conclusion: This innovative surgical technique is particularly effective for complex cases requiring simultaneous occlusion of the IVC and both RVs. It enhances patient safety and outcomes and has significant potential for broader application in urology and general surgery. This case highlights the value of surgical innovation in overcoming complex challenges and provides valuable insights for the medical community.

Dysregulated specificity proteins (SPs), members of the C2H2 zinc-finger family, are crucial transcription factors (TFs) with implications for renal physiology and diseases. This comprehensive review focuses on the role of SP family members, particularly SP1 and SP3, in renal physiology and pathology. A detailed analysis of their expression and cellular localization in the healthy human kidney is presented, highlighting their involvement in fatty acid metabolism, electrolyte regulation, and the synthesis of important molecules. The review also delves into the diverse roles of SPs in various renal diseases, including renal ischemia/reperfusion injury, diabetic nephropathy, renal interstitial fibrosis, and lupus nephritis, elucidating their molecular mechanisms and potential as therapeutic targets. The review further discusses pharmacological modulation of SPs and its implications for treatment. Our findings provide a comprehensive understanding of SPs in renal health and disease, offering new avenues for targeted therapeutic interventions and precision medicine in nephrology.

Renal cell carcinomas, which comprise the majority of primary kidney tumors, present diverse challenges in treatment planning. This case report explores the application of simultaneous partial nephrectomy and radiofrequency (RF) ablation in a patient with a solitary kidney, emphasizing the significance of specific interventions. A 52-year-old female patient who underwent radical nephrectomy presented with right flank pain and hematuria. Contrast-enhanced computed tomography revealed exophytic and calyceal system-extending lesions in the right kidney. A multidisciplinary approach involving interventional radiology and nephrology facilitated preoperative preparation. The patient underwent simultaneous partial nephrectomy and RF ablation in a single session, resulting in favorable outcomes. This meta-analysis highlighted radical nephrectomy's association with chronic kidney disease, emphasizing the need for specialized treatment approaches. Ablative treatment was associated with superior perioperative outcomes, whereas partial nephrectomy exhibited higher urological complication rates. Minimally invasive techniques are crucial, especially for solitary kidney cases and small renal tumors. Simultaneous partial nephrectomy and RF ablation are effective for managing exophytic and calyceal system-extending masses in solitary kidneys. The preservation of renal function is of paramount importance, prompting consideration of ablative treatment alongside partial nephrectomy. Despite limited evidence, ablative therapies offer a viable alternative for frail and comorbid patients, ensuring long-term oncological durability and superior preservation of renal function. Keywords:Ablation, partial nephrectomy, radical nephrectomy, radiofrequency ablation, renal cell carcinoma, renal tumor, tumor: [ABSTRACT FROM AUTHOR]

Background: This study aimed to identify disease‐causing variants within a Chinese family affected by Birt–Hogg–Dubé syndrome (BHDS), which arises from an autosomal dominant inheritance pattern attributed to variants in the folliculin (FLCN) gene, recognized as a tumor suppressor gene. Methods: A Chinese proband diagnosed with BHDS due to renal tumors underwent next‐generation sequencing (NGS), revealing a novel variant in the FLCN gene. Sanger sequencing was subsequently performed on blood samples obtained from family members to confirm the presence of this variant. Results: A novel germline frameshift variant (NM_144997.5:c.977dup) was identified in five individuals among the screened family members, marking the first report of this variant. Additionally, a somatic frameshift variant (NM_144997.5:c.1252del) was detected in the renal tumors of the proband. No variant was detected in unaffected family members. Conclusions: A novel heterozygous variant was identified in exon 9 of the FLCN gene, which broadens the spectrum of FLCN variants. We recommend that molecular analysis of the FLCN gene be performed in patients with suspected BHDS and their families. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Computer vision technology is more and more widely used in the market. Target detection and feature extraction are two important auxiliary means of this technique, which are helpful to analyze target motion data. However, in the field of biology, there are some data limitations in the analysis of targets such as bacteria and tumors, which need to be further explored. Optical MRI imaging technology based on computer vision provides a new way to extract and analyze morphological features of renal tumors. In this paper, an optical MRI imaging method based on computer vision is designed and developed for the extraction and analysis of morphological features of kidney tumors. By using optical MRI imaging technology based on computer vision, the morphological characteristics of kidney tumors were extracted by analyzing the optical characteristics and MRI images of kidney tumors, and a simulation model was established to simulate the morphological characteristics of different types of kidney tumors, and feature extraction and analysis were carried out by computer algorithm. Through the analysis of the simulation model, the morphological characteristics of renal tumors were extracted and analyzed, which provided a new and non-invasive method for clinical diagnosis and treatment of renal tumors.

Glomus tumors are rare mesenchymal tumors involving cells from the glomus body, smooth muscle, and vasculature, typically found in distal extremities' skin. This case describes a 54-year-old woman with a history of hypothyroidism and hyperlipidemia, incidentally discovered to have a four-centimeter calcified renal tumor. Surgery was performed due to suspected malignancy. Immunohistochemical staining confirmed a renal glomus tumor, positive for muscle actin and smooth muscle actin (SMA). The tumor was benign, and no adjuvant therapy was needed. The patient remained recurrence-free during follow-up. Renal glomus tumors are predominantly benign, with surgical resection as the primary treatment.

Objective: This study aims to report a renal tumor in a horseshoe kidney successfully treated with an open left nephrectomy. Case(s) Presentation: A 3 years old girl with a lump in the left abdomen and intermittent hematuria was referred to our hospital. Whole abdominal contrast CT scan showed the presence of a left renal mass in a horseshoe kidney that infiltrates the left renal artery and is attached to the spleen. The patient underwent preoperative chemotherapy followed by surgical resection. Discussion: In a horseshoe kidney, renal tumors are uncommon. The limited kidney mobilization and access to the renal hilum and the abnormal and highly variable vasculature make surgical treatment of a tumor in a horseshoe kidney extremely difficult. Conclusion: Open surgery continues to be the treatment of choice for horseshoe kidney tumors due to their anatomical complexity, particularly in cases where the tumor is difficult to eradicate. Keywords: Renal tumor, horseshoe kidney, surgical management.

The use of 3D laparoscopic partial nephrectomy has emerged as a cornerstone in the surgical arsenal for addressing renal tumors, particularly in managing challenging cases characterized by deeply seated tumors embedded within the renal parenchyma. In these intricate scenarios, the utilization of intraoperative ultrasound (IOUS) acquires paramount importance, serving as an indispensable tool for guiding and meticulously monitoring the surgical process in real time. To further explore the efficacy of IOUS-guided techniques, we conducted a retrospective study comparing outcomes in patients who underwent partial nephrectomy with IOUS guidance (n = 60) between 2020 and 2022 with a cohort from 2018 to 2019 without IOUS guidance (n = 25). Our comprehensive analysis encompassed various post-operative parameters, including the duration until food resumption, analgesia requirements, and length of the hospital stay. While these parameters exhibited comparable outcomes between the two groups, notable distinctions emerged in the intraoperative metrics. The IOUS-guided cohort demonstrated significantly reduced blood loss, a shorter median operative duration, and diminished ischemia time (p = 0.001). These compelling findings underscore the undeniable benefits of IOUS-guided techniques in not only facilitating the attainment of negative surgical margins but also in enhancing procedural safety and precision, thereby contributing to improved patient outcomes in the management of renal tumors. [ABSTRACT FROM AUTHOR]

Wilms tumor (WT) occurring in adults is rare and even much more rarely found to coexist with pregnancy. Clinical outcome in adults is worse overall compared with pediatric patients with WT and is often misdiagnosed with no standardized protocols for care guided by high‐evidence clinical trials. We present a case of a 23‐year‐old woman diagnosed with WT who was found to be pregnant immediately following nephrectomy. Workup findings showed that she had disseminated disease but was successfully managed in a multidisciplinary team setting with modified intrapartum chemotherapy followed by postpartum chemotherapy. In low‐resource settings, management protocols for adult patients with WT can be individualized by multidisciplinary teams to leverage available resources for best outcomes. Synopsis: Disseminated adult Wilms tumor, a rare and challenging condition in pregnancy, can be successfully managed in a low‐resource setting by a multidisciplinary team. [ABSTRACT FROM AUTHOR]

Objective. Laparoscopic renal unit-preserving resection is a routine and effective means of treating renal tumors. Image segmentation is an essential part before tumor resection. The current segmentation method mainly relies on doctors manual delineation, which is time-consuming, labor-intensive, and influenced by their personal experience and ability. And the image quality of segmentation is low, with problems such as blurred edges, unclear size and shape, which are not conducive to clinical diagnosis. Approach. To address these problems, we propose an automated segmentation method, i.e. the UNet++ algorithm fusing multiscale residuals and dual attention (MRDA_UNet++). It replaces two consecutive 3 × 3 convolutions in UNet++ with the 'MultiRes block' module, which incorporates coordinate attention to fuse features from different scales and suppress the impact of background noise. Furthermore, an attention gate is also added at the short connections to enhance the ability of the network to extract features from the target area. Main results. The experimental results show that MRDA_UNet++ achieves 93.18%, 92.87%, 93.66%, and 92.09% on the real-world dataset for MIoU, Dice, Precision, and Recall, respectively. Compared to the baseline model UNet++ on three public datasets, the MIoU, Dice, and Recall metrics improved by 6.00%, 7.90% and 18.09% respectively for BUSI, 0.39%, 0.27% and 1.03% for Dataset C, and 1.37%, 1.75% and 1.30% for DDTI. Significance. The proposed MRDA_UNet++ exhibits obvious advantages in feature extraction, which can not only significantly reduce the workload of doctors, but also further decrease the risk of misdiagnosis. It is of great value to assist doctors diagnosis in the clinic. [ABSTRACT FROM AUTHOR]

Objective: Complete endophytic renal tumors (CERTs) are the most challenging for robot-assisted partial nephrectomy (RAPN). This study aimed to determine the impact of CERT on outcomes of RAPN. Methods: All RAPN cases for localized renal tumor undertaken at Yokohama City University Hospital between 2016 and 2023 were enrolled. Tumor characteristics and surgical, functional, and oncologic outcomes of RAPN were compared between CERT and non-CERT groups. Results: Consecutive 666 patients were enrolled, and 76 (11.4%) were identified as CERT (3 points of "E" score). CERT showed smaller tumor diameters (p < 0.001), more predominant hilar tumor (p = 0.029), higher "N" scores (p < 0.001) and "L" scores (p = 0.006) than non-CERT. The CERT group showed longer warm ischemia times (p < 0.001), more frequent positive surgical margins (p = 0.028), and relatively lower trifecta achievement rates (p = 0.101) than the non-CERT group. In multivariable analysis, the CERT was an independent predictor for trifecta achievement but not for pentafecta achievement. Conclusions: CERT was associated with longer warm ischemia time, positive surgical margin, and lower trifecta achievement, but not with surgical complication and pentafecta achievement in RAPN. This study suggested that CERT had limited influence on long-term renal functional preservation; however, it had strong impacts on short-term surgical outcome. [ABSTRACT FROM AUTHOR]

We retrospectively collected baseline, perioperative and follow up data regarding RAPN and MWA from a single academic center. MWA carried a lower burden for the patient in terms of length of stay and medical complications, though it resulted in a higher rate of recurrence. Renal function variation was comparable after the two procedures. Background: Robot assisted partial nephrectomy (RAPN) and microwave ablation (MWA) are 2 of the most advanced techniques for the management of localized small renal masses. Purpose: To compare the perioperative, functional and oncological results of RAPN and MWA. Materials and Methods: Data from 171 consecutive patients undergoing either RAPN or MWA for a localized small renal mass at a single academic center was retrospectively collected. Baseline features included patients' demographics and masses' characteristics. Procedures were compared in terms of perioperative outcomes and renal function variation Progression of a persistent lesion or local recurrence after a complete treatment defined local tumor progression. Descriptive statistics and survival analysis tested the association between predictors and local tumor progression. Results: Of all, 109 and 62 patients underwent RAPN and MWA. Patients in the MWA group were older (P = .002) had higher Charlson Comorbidity Index (CCI) (P < .001) and higher frequency of preoperative chronic kidney disease (P < .001). MWA led to a shorter postoperative hospitalization time (P < .001) and lower incidence of medical complications (6.5% vs. 22.9%, P = .02) than RAPN. GFR decline was similar between groups both at discharge (P = .39) and at the time of last follow up (P = 1.00). A lower rate of secondary interventions (11.7% vs. 2.8%, P = .037) and a better disease-free survival (83.2% vs. 96.5%, P = .027) were reported after RAPN. Conversely, cancer specific and overall survival were comparable (P > .05). At univariate regression analysis, MWA was associated with local tumor progression (HR 3.46, P = .040). Conclusion: MWA displayed a lower perioperative impact, while functional outcomes were similar after each intervention. RAPN resulted superior in terms of tumor eradication, but no difference was noted regarding cancer specific survival. Thus, MWA represents a valid alternative in frail patients, though less radical than RAPN. [ABSTRACT FROM AUTHOR]

Introduction: The most common sites of clear cell renal cell carcinoma(ccRCC) metastasis are the lung, bones, liver and brain; eyelid metastasis is a rare occurrence. Case presentation: We report a case of ccRCC metastasis to the left eyelid after radical nephrectomy, and remission after sunitinib treatment. Conclusions: Although the probability of eyelid metastasis rate is very low, tumor metastasis to the eyelid skin is possible after radical nephrectomy. Therefore, any rash like changes on the skin during the review procedure cannot be ignored by the physician. [ABSTRACT FROM AUTHOR]

Thauvin‐Robinet‐Faivre syndrome (#617107) is a rare autosomal recessive overgrowth syndrome characterized by intellectual disability, facial dysmorphism, macrocephaly, and variable congenital malformations. It is caused by homozygous or compound heterozygous FIBP gene mutations. The FIBP gene is located on the 11q13.1 region and codes the acidic fibroblast growth factor intracellular binding protein, which is involved in the fibroblast growth factor (FGF) signaling pathway. FGF signaling is required for neurogenesis and neuronal precursor proliferation. The FGF controls cell proliferation, differentiation, and migration in embryonic development and in adult life. Overgrowth syndromes consist of a wide spectrum disorders characterized by prenatal and postnatal excess growth in weight and length, often associated malformations, intellectual disability, and neoplastic predisposition. Embryonic tumors are especially common in these syndromes. Thauvin‐Robinet‐Faivre syndrome is a recently described overgrowth syndrome with typical facial dysmorphic and clinical features. To date, only four patients have been reported with this disorder. Herein, two new cases of Thauvin‐Robinet‐Faivre syndrome are reported with overgrowth, intellectual disability, typical dysmorphic signs in one dysplastic kidney, and a novel homozygous FIBP gene variant. Exome sequencing analysis showed that both affected siblings share the same homozygous c. 412‐3_415dupCAGTTTG FIBP gene variant. Reporting two new cases with this rare autosomal recessive overgrowth syndrome with a novel FIBP gene variant will support and expand the clinical spectrum of Thauvin‐Robinet‐Faivre syndrome. Also discussed will be the function of FIBP in tumorigenesis and the possible renal tumor susceptibility in heterozygous carriers will be emphasized. [ABSTRACT FROM AUTHOR]

Synovial sarcomas are a rare and aggressive subtype of soft tissue sarcomas that typically affects young adults and involves the extremities. Synovial sarcoma of the kidney is a rare and aggressive tumor with a poor prognosis, accounting for only 1% of all renal tumors. The imaging features of this tumor often overlap with those of other renal tumors, and a definitive diagnosis can only be made through immunohistochemical analysis. In this case report, we present the case of a 55-year-old female with left flank pain, who was diagnosed with primary renal synovial sarcoma following a left-sided radical nephrectomy. Despite initial successful surgical intervention, restaging scans showed local recurrence and metastatic disease, which was subsequently managed with 6 cycles of chemotherapy followed by radiation therapy with palliative intent. This case underscores the importance of early detection and aggressive management of rare renal tumors to improve patient outcomes.

Background: Surgical removal of huge renal and retroperitoneal tumors, such as adrenal cortical carcinoma or renal cell carcinoma with inferior vena cava (IVC) invasion, remains a challenge for urologists. Aim: Herein, we describe our experience with the modified Makuuchi incision for huge renal and retroperitoneal tumors. Methods: We applied the modified Makuuchi incision in 10 patients with a huge renal or retroperitoneal tumor. Another 11 patients with a huge renal or retroperitoneal tumor using different surgical incisions other than modified Makuuchi incision were collected for comparison. The modified Makuuchi incision of the abdomen is initiated in the cephalad to the xiphoid, extended 1 cm above the umbilicus, and then extended laterally to the lateral flank. Through the incision, we mobilized the colon, and when the renal space was seen, the tumor was removed. If an invasion of a nearby organ occurred, a general or cardiovascular surgery specialist was consulted for combined surgery. The patients' age ranged from 43 to 82 years (three men and seven women). Results: The modified Makuuchi incision provided good and rapid exposure. No unexpected organ injuries occurred during surgery. There were no wound-related complications, such as dehiscence or incisional hernia, after 3 months of follow-up. Partial hepatectomy, splenectomy, distal pancreatectomy, and thrombectomy of the IVC thrombus were performed through the same incision. Conclusion: Our experience demonstrated that the modified Makuuchi incision is a good choice for patients with huge renal and retroperitoneal tumors, even those with nearby organ invasion.

Renomedullary interstitial cell tumor (RMICT), referred to as a medullary fibroma, is almost always asymptomatic and incidentally identified either at autopsy or upon resection of the kidney for other reasons. Although a few cases of RMICTs that are large in size and clinically symptomatic have been reported, there are few reports of RMICTs contrasting imaging findings with pathological findings. In this report, we describe a relatively large RMICT case of 3 cm in size, focusing on the radiologic-pathologic correlation.

The aim of this study is to compare perioperative parameters of laparoscopic partial nephrectomy and open partial nephrectomy in renal tumor management, and to evaluate the effect of using novel method of indocyanine green navigation in segmental ischemia on these parameters. The prospective study included 455 patients (89 laparoscopic partial nephrectomies, 366 open partial nephrectomies). Sub-groups (n=39, 32, 18) in Laparoscopic partial nephrectomy employed diverse ischemia techniques, including full warm ischemia, segmental ischemia with indocyanine green navigation and segmental ischemia without navigation. Parameters assessed encompassed estimated blood loss, ope­rative time, warm ischemia time, and changes in estimated glomerular filtration rate. Covariate-balancing propensity scores ensured homogeneity. Statistical analysis included the Wilcoxon signed-rank test, for two matched groups. Two-sided p-values were reported for all statistical tests, a p-value

Objective To investigate the feasibility and safety of modified enhanced recovery after surgery (ERAS) in laparoscopic partial nephrectomy. Methods A retrospective analysis was conducted with the clinical data of renal tumor patients treated with laparoscopic partial nephrectomy in the Urology Department of Peking Union Medical College Hospital from May 2019 to June 2021. According to the peri-operative ERAS strategy, they were divided into modified ERAS group and conventional group. The postoperative recovery and complications of the two groups were compared. Results A total of 243 patients were enrolled, including 142 in the modified ERAS group and 101 in the conventional group. There was no significant difference between the two groups in baseline of indicators such as region, age, gender and tumor stage. The time records of first drinking water, the time of first exhaust, the first off-bed movement, catheter removal and drainage tube removal, the post-operative hospital stay and the total expense of hospitalization in the improved ERAS group were significantly superior to those in the conventional group (all P＜0.05). Conclusions The application of improved ERAS during the peri-operative period of laparoscopic partial nephrectomy may accelerate post-operative recovery.

Objective To study the expression of long-chain non-coding RNA (lncRNA) RP11-626G11.3 in renal cancer tissues and cell lines, and to explore the effect of knockdown of RP11-626G11.3 on the biological behavior of renal cancer cells. Methods The GEPIA database was used to analyze the expression of RP11-626G11.3 in renal cancer tissues and adjacent tissues, and the TCGA database was used to analyze the relationship between the expression of RP11-626G11.3 and the survival time of renal cancer patients. The expression of RP11-626G11.3 in various renal cancer cell lines was detected by qPCR. The renal cancer cells with the highest expression of RP11-626G11.3 were selected and transfected with control plasmid (si-NC group) and RP11-626G11.3 silencing plasmid (si-RP11-626G11.3 group). Cell proliferation and migration were examined by MTT assay and cell scratch assay. The microRNA (miRNA) binding to RP11-626G11.3 was found by bioinformatics method, and verified by dual-luciferase reporter gene experiment. The expression of miR-532-3p in the two groups of cells was detected by qPCR. Western blot was used to detect the expression levels of Wnt/β-catenin signaling pathway in the two groups of cells. Results Compared with adjacent tissues, the expression of RP11-626G11.3 was up-regulated in renal cancer tissues (P＜0.01). Compared with patients with high expression of RP11-626G11.3, patients with low expression of RP11-626G11.3 had a longer survival time (P＜0.01). Compared with immortalized renal tubular epithelial cells, the expression of RP11-626G11.3 was up-regulated in renal cancer cell lines (P＜0.01), and the expression of RP11-626G11.3 was the highest in OS-RC-2 cells (P＜0.01). Compared with si-NC group, the viability of OS-RC-2 cells in si-RP11-626G11.3 group was significantly decreased (P＜0.05) with decreased cell migration rate(P＜0.01). RP11-626G11.3 was found to target at miR-532-3p (P＜0.01). Compared with the si-NC group, the expression of miR-532-3p in OS-RC-2 cells in the si-RP11-626G11.3 group was significantly up-regulated (P＜0.01), and the Wnt/β-catenin signaling pathway proteins β-catenin, Axin2, C-myc, cyclin D1 and MMP7 decreased. Conclusions The expression of RP11-626G11.3 is increased in renal cancer tissues and cell lines. Knockdown of RP11-626G11.3 inhibits the proliferation and migration of renal cancer cells by regulating the expression of miR-532-3p.

Mixed epithelial and stromal tumor (MEST) of the kidney is a rare benign tumor with malignant potential, and is characterized by epithelial and stromal proliferation with a variety of cellularity and growth pattern. MEST of the kidney is often depicted as a well-defined, solid mass with a cystic component. However, due to the rarity of the disease, there are no reports of its progression in serial imaging examinations. This report presents the case of a 68-year-old woman with MEST who was followed for 13 years by computed tomography (CT). To the best of our knowledge, this is the first report of image findings of MEST of the kidney over a follow-up period longer than 10 years.

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.

Ossifying Renal Tumor of Infancy (ORTI) represents an extremely rare and benign renal neoplasm, with limited cases published in the literature. Predominantly characterized by painless and intermittent gross hematuria, the diagnostic evaluation is effectively facilitated through ultrasound, computed tomography, and magnetic resonance imaging. Despite progress, its etiology has not yet been elucidated. We report an additional case with an unusual clinical presentation.

To investigate the potential of amide proton transfer-weighted (APTw) MRI in identifying benign and malignant renal tumors and to evaluate whether APTw MRI can add diagnostic value to diffusion-weighted imaging (DWI). Participants with renal tumor underwent preoperative multiparametric MRI, including APTw MRI and DWI. The APTw and apparent diffusion coefficient (ADC) of malignant tumors and benign tumors were calculated independently by two radiologists and compared. The value of the mean APTw and the mean ADC for differentiating malignant and benign tumors was evaluated by receiver operating characteristic analysis. In total, 65 participants (mean age, 59 years ±14; 41 men) were evaluated: 54 with malignant and 11 with benign renal tumors. Malignant renal tumors showed higher mean APTw values [2.03% (1.63) vs 1.00% (1.60); P < 0.01] and lower mean ADC values (1.22 × 10-3 mm2/s ± 0.37 vs 1.51 × 10-3 mm2/s ± 0.37; P < 0.05) than benign renal tumors. The area under the receiver operating characteristic curve (AUC) of APTw, ADC and the combination of them for the identification of benign and malignant renal tumors was 0.78(95% CI: 0.66, 0.87; P < 0.001),0.70(95% CI: 0.54, 0.86; P < 0.05) and 0.79 (95% CI: 0.67, 0.88; P < 0.001). The optimal cutoff value for mean APTw was 2.14% (sensitivity, 74%; specificity, 73%). There was no difference between these three parameters for differentiating malignant from benign renal tumors (P > 0.05). The APTw MRI has the potential use as an imaging biomarker for renal malignant and benign tumors. [ABSTRACT FROM AUTHOR]