Renal Malignancy Research Articles

Multifunctional intracellular matrix metalloproteinases: implications in disease.

Matrix metalloproteinases (MMPs) are zinc-dependent endopeptidases that were first discovered as proteases, which target and cleave extracellular proteins. During the past 20 years, however, intracellular roles of MMPs were uncovered and research on this new aspect of their biology expanded. MMP-2 is the first of this protease family to be reported to play a crucial intracellular role where it cleaves several sarcomeric proteins inside cardiac myocytes during oxidative stress-induced injury. Beyond MMP-2, currently at least eleven other MMPs are known to function intracellularly including MMP-1, MMP-3, MMP-7, MMP-8, MMP-9, MMP-10, MMP-11, MMP-12, MMP-14, MMP-23 and MMP-26. These intracellular MMPs are localized to different compartments inside the cell including the cytosol, sarcomere, mitochondria, and the nucleus. Intracellular MMPs contribute to the pathogenesis of various diseases. Cardiovascular renal disorders, inflammation, and malignancy are some examples. They also exert antiviral and bactericidal effects. Interestingly, MMPs can act intracellularly through both protease-dependent and protease-independent mechanisms. In this review, we will highlight the intracellular mechanisms of MMPs activation, their numerous subcellular locales, substrates, and roles in different pathological conditions. We will also discuss the future direction of MMP research and the necessity to exploit the knowledge of their intracellular targets and actions for the design of targeted inhibitors.

Infiltrative Renal Malignancies: Imaging Features, Prognostic Implications, and Mimics.

Infiltrative renal malignancies are a subset of renal masses that are morphologically characterized by a poorly defined interface with the renal parenchyma. Infiltrative renal malignancies are less common but more aggressive than more typical renal malignancies and carry an overall worse prognosis. Although an infiltrative renal process often represents a malignant neoplasm, infiltrative masses include a wide spectrum of diseases including primary renal cortical, medullary, and pelvic tumors; lymphoproliferative processes; metastases; and various infectious, inflammatory, immune-mediated, and vascular mimics. The imaging features of these masses are often nonspecific, but with the appropriate history, laboratory results, and clinical context, the radiologist can help narrow the diagnosis and guide further treatment. An invited commentary by Lee is available online.Online supplemental material is available for this article. ©RSNA, 2021.

Wilms' Tumor Primary Cells Display Potent Immunoregulatory Properties on NK Cells and Macrophages.

Simple SummaryWilms’ tumor (WT) is the most common childhood renal tumor accounting for approximately 7% of childhood malignancies. The overall survival rate for patients with favorable histology is greater than 90% while the survival rate for patients with poor prognostic factors is around 50%. The current treatments consist in a combination of surgery and chemotherapy or radiotherapy in high risk patients. Such treatments are responsible for significant adverse effects requiring long-term monitoring. Thus, a main challenge in WT treatment is the development of novel therapeutic strategies to eliminate or minimize the adverse effects. The characterization of an immune environment could allow the identification of new therapeutic targets. Herein we studied the interaction between WT and innate immune cells, in particular NK cells and monocytes. Although WT are highly susceptible to NK-mediated lysis, the detection of immunoregulatory activity of WT tumor cells on NK cells and also on monocytes could offer novel cellular and molecular targets for an efficacious immunotherapy of WT.The immune response plays a crucial defensive role in cancer growth and metastasis and is a promising target in different tumors. The role of the immune system in Wilm’s Tumor (WT), a common pediatric renal malignancy, is still to be explored. The characterization of the immune environment in WT could allow the identification of new therapeutic strategies for targeting possible inhibitory mechanisms and/or lowering toxicity of the current treatments. In this study, we stabilized four WT primary cultures expressing either a blastematous (CD56+/CD133−) or an epithelial (CD56−/CD133+) phenotype and investigated their interactions with innate immune cells, namely NK cells and monocytes. We show that cytokine-activated NK cells efficiently kill WT cells. However, after co-culture with WT primary cells, NK cells displayed an impaired cytotoxic activity, decreased production of IFNγ and expression of CD107a, DNAM-1 and NKp30. Analysis of the effects of the interaction between WT cells and monocytes revealed their polarization towards alternatively activated macrophages (M2) that, in turn, further impaired NK cell functions. In conclusion, we show that both WT blastematous and epithelial components may contribute directly and indirectly to a tumor immunosuppressive microenvironment that is likely to play a role in tumor progression.

The Differential Diagnosis of Medullary-Based Renal Masses.

Renal malignancies can be divided into cortical- and medullary-based tumors, the latter of which classically infiltrate the renal parenchyma by extending between nonneoplastic structures. Although high-grade cortical tumors can rarely exhibit the same growth pattern, the infiltrative morphology should elicit a differential diagnosis to be considered in each case. However, these diagnoses can be challenging to distinguish, especially on small renal biopsy samples. To provide an overview of the clinical, gross, and microscopic findings; genetic and molecular alterations; and immunohistochemical evaluation of medullary-based renal tumors and other tumor types with overlapping morphologies and growth patterns. Literature review and personal observations were used to compile the information in this review. Collecting duct carcinoma is a prototypical medullary-based tumor, and although diagnostic criteria exist, it remains a diagnosis of exclusion, especially with ancillary techniques aiding the recognition of established as well as more recently described neoplasms. Other medullary-based malignancies included in the differential diagnosis include renal medullary carcinoma/renal cell carcinoma unclassified with medullary phenotype, fumarate hydratase-deficient renal cell carcinoma, and upper tract urothelial carcinoma. Moreover, other rare entities should be excluded, including metastatic carcinoma, lymphoma, and melanoma. In addition to potential prognostic differences, accurate diagnoses can have important surgical and clinical management implications.

Percutaneous thermoablation of small renal masses (T1a) in surgical candidate patients: oncologic outcomes.

To evaluate the local tumour progression-free survival (LTPFS), metastasis-free survival (MFS), cancer-specific survival (CSS) and overall survival (OS) of healthy surgical candidates who underwent percutaneous thermoablation (TA) as a first-line therapy for small renal masses (T1a). The institutional review board approved this bi-institutional retrospective study of 85 consecutive surgical candidates with 97 biopsy-proven malignant renal masses (T1a) treated with percutaneous TA from 2008 to 2016. The LTPFS, MFS, CSS and OS rates were calculated using the Kaplan-Meier method. Descriptive analysis was also performed. The median tumour size was 2.3 cm (range, 0.7-3.9 cm). The minimal and mean follow-up periods were 24 and 56 months, respectively. Local recurrence was detected in four patients (4.7%) at 8.5, 13.8, 58.0 and 64.0 months of follow-up and retreated successfully with percutaneous TA. No patient developed metastatic renal cell carcinoma, and none died due to renal oncologic complications. One patient died of heart attack. The 5-year LTPFS, OS, MFS and CSS rates were 93.0%, 98.4%, 100% and 100%, respectively. Only two patients (2.3%) had major complications (Clavien-Dindo grade > II), including ureteropelvic junction stenosis and urinary obstruction due to ureteral blood clots. Our study demonstrates that percutaneous TA is a feasible and effective first-line therapy for healthy surgical candidates with small renal masses (T1a). The 5-year LTPFS, OS, CSS and MFS rates were 93.0%, 98.4%, 100% and 100%, respectively, with a major complication rate of only 2.3%. • Image-guided percutaneous thermoablation of small renal malignancies was effective in 95.3% of the healthy surgical candidates. • Major complications were detected in 2.3% of the patients. • The local tumour progression-free survival rate was 97.6% and 93.0% at 3 and 5 years, respectively.

Clinicopathological Features, Management and Outcome of Paediatric Solid Renal Tumours in Southeast Nigeria: The Need for Protocol and Multidisciplinary Collaboration

Introduction: Renal malignancies are common in children and they constitute 6-7% of all childhood tumours and nephroblastoma is the most common solid renal tumour in children. Currently, standardised institutional protocols in management of renal tumours in children are the norm. Large scale collaborative studies have started emerging, yet not much has been documented on the clinical presentation, pathology and outcome of solid renal tumours particularly in Africa. Aim: To review the presentations, pathology and the management-outcome of solid renal tumour in the centre in the absence of a coordinated protocol and multi-disciplinary collaboration. Materials and Methods: This was a nine and a half year longitudinal retrospective audit study of consecutive patients with solid renal tumours managed in a single tertiary centre: Nnamdi Azikiwe University Teaching Hospital Nnewi South-east Nigeria. It took place from January 2009-June 2018. Relevant data on demography, clinical features, management and outcome were extracted from records. Results were analysed using Statistical Package for Social Sciences (SPSS) version 22. Categorical data were tested for independence using Chi-square test and significant p-value set at <0.05. Results: Twenty two paediatric cases (15 males and 7 females) were included in the study. The mean age at presentation was 50.10±4.8 months. There were 15 males and 7 females. The mean duration of symptoms was 5.5 months; 21 (95%) presented with abdominal masses while 6 (27.3%) had gross haematuria and 13 (59%) were emaciated. Tumours involved left kidney in 15 (68%). Histological reports were available in 9 cases with nephroblastoma being the most common 6 (27.3%). The Commonest stage was stage 3, 10 (45%) and commonest procedure was nephroureterectomy, 11 (50%). Neo-adjuvant and adjuvant therapies were inconsistent. Conclusion: Late presentation, discordant treatment protocol, noncompletion of treatment and poor collaboration, were found to contribute largely to poor outcome of solid renal tumours in children in our setting. It is strongly believed that adoption of standard protocol and a multidisciplinary collaboration in management will improve records keeping and outcome.

The Systemic Inflammation on Transradial vs Transfemoral Approach for Coronary Angiography in Patients with Chronic Coronary Syndrome <br/>—Does Transradial Coronary Angiography Trigger More Inflammation?

Background: Most of the institutions accept the Transradial Access (TRA) as the first approach for patients undergoing Coronary Angiography (CAG) and Percutaneous Coronary Interventions (PCI). Several studies clearly revealed endothelial injury of coronary arteries triggers inflammatory response. In this study, we aimed to evaluate inflammatory respond to CAG and to compare the inflammatory response of TRA and Transfemoral Access (TFA). Methods: In this single-center prospective study 140 consecutive patients presenting with (Chronical Coronary Syndrome) CCS and underwent transradial or transfemoral CAG between December 2017 and December 2018 were included. After exclusions, left 92 patients were divided into two equal groups as TRA and TFA. The primary endpoints were 2nd hour Tumor Necrosis Factor alpha (TNFα), 48th hour high-sensitivity C-Reactive Protein (hs-CRP), complication rates, amount of contrast medium, procedure time and fluoroscopy time. Results: Basal characteristics of TRA and TFA groups were similar. A comparison of variables demonstrated that there was no statistical significance in increase in inflammatory markers (TNFα, hs-CRP), complication rates, amount of contrast media and procedure time. In contrast, TRA was associated longer fluoroscopy time and higher X-ray dosage, although statistically insignificant. CAG caused a similar increase in hs-CRP in both groups, insignificantly. Conclusion: In contrast to previous studies reporting a higher inflammatory response with TRA, this study demonstrates that inflammatory response caused by CAG is unrelated to access site and similar in TRA and TFA. Longer fluoroscopy time, higher X-ray dosage and longer procedure time in the TRA group indicate the importance of carefully selecting the angiographic route especially in patients with chronic heart failure, chronic renal impairment and malignancy risk. Also, these parameters place importance for physicians own health and woman planning pregnancy.

Extremely elevated erythrocyte sedimentation rate: patient diagnoses at a tertiary Indian hospital

Introduction and Aim: Extremely elevated Erythrocyte Sedimentation Rate (EEESR) is the term commonly used for elevation of ESR more than or equal to 100mm in the first hour. To the best of our knowledge, this is the first study in Indian population. Aim was to study the common etiologies of EEESR in Indian patients at a tertiary hospital.
 Materials and Methods: A retrospective, cross- sectional and descriptive study was carried out and included all patients 18 years of age and above, and who had a raised ESR of 100 mm and above at the end of 1st hour at least one time between January 2018 to December 2018 and tested at our institute. The patient’s demographic details, ESR values and diagnoses were retrieved from electronic records and were divided into 7 categories like infections, malignancy, renal diseases, autoimmune diseases, mixed diagnoses, miscellaneous and unknown.
 Results: During the study period, 607 patients had EEESR. There were 335 males and 272 females. The mean age was 55 years. Within the broad categories of diseases, infection was the most common cause (52%) followed in descending order by renal diseases (12.4%), malignancy (9.6%), miscellaneous (8.4%), mixed diagnoses (7.6%), autoimmune diseases (7%) and unknown (3%). In the infection category, pneumonia was the commonest.
 Conclusion: This study on frequency of common etiologies of EEESR is the first study in Indian population. Our study reaffirms findings that most patients have an underlying cause for EEESR. The commonest etiology in Indian patients is infection with pneumonia being the frequent cause.

Strategic management of adult wilms' tumor: A case analysis of single-institution experience and review of literatures

Background: Wilms' tumor (nephroblastoma) is the most common solid primary renal malignancy in children but extremely rare in adult population. We report the clinical presentation and therapeutic results of our adult Wilms' tumor patients and review the previous literatures about diagnosis and treatments. Methods: A retrospective review was carried out to investigate the patients with adult Wilms' tumor at the Tri-Service General Hospital during a 35-year period (1980–2014). Seven patients ≥16 years old (one female and six males; median age at the time of diagnosis of 24 years; range: 21–48 years) were staged according to the National Wilms' Tumor Study staging system as follows: three patients had Stage II disease, two patients had Stage III disease, and two patients had Stage IV disease. All statistical values were represented as mean ± standard deviation. All tests were two-sided, with P

Differentiation between Clear Cell Sarcoma of the Kidney and Wilms' Tumor with CT.

ObjectiveClear cell sarcoma of the kidney (CCSK) is the second-most common but extremely rare primary renal malignancy in children after Wilms' tumor. The aims of this study were to evaluate the imaging features that could distinguish between CCSK and Wilms' tumor and to assess the features with diagnostic value for identifying CCSK.Materials and MethodsWe reviewed the initial contrast-enhanced abdominal-pelvic CT scans of children with CCSK and Wilms' tumor between 2010 to 2019. Fifty-eight children (32 males and 26 females; age, 0.3–10 years), 7 with CCSK, and 51 with Wilms' tumor, were included. The maximum tumor diameter, presence of engorged perinephric vessels, maximum density of the tumor (Tmax) of the enhancing solid portion, paraspinal muscle, contralateral renal vein density, and density ratios (Tmax/muscle and Tmax/vein) were analyzed on the renal parenchymal phase of contrast-enhanced CT. Fisher's exact tests and Mann-Whitney U tests were conducted to analyze the categorical and continuous variables, respectively. Logistic regression and receiver operating characteristic curve analyses were also performed.ResultsThe age, sex, and tumor diameter did not differ between the two groups. Engorged perinephric vessels were more common in patients in the CCSK group (71% [5/7] vs. 16% [8/51], p = 0.005). Tmax (median, 148.0 vs. 111.0 Hounsfield unit, p = 0.004), Tmax/muscle (median, 2.64 vs. 1.67, p = 0.002), and Tmax/vein (median, 0.94 vs. 0.59, p = 0.002) were higher in the CCSK compared to the Wilms' group. Multiple logistic regression revealed that engorged vessels (odds ratio 13.615; 95% confidence interval [CI], 1.770–104.730) and Tmax/muscle (odds ratio 5.881; 95% CI, 1.337–25.871) were significant predictors of CCSK. The cutoff values of Tmax/muscle (86% sensitivity, 77% specificity) and Tmax/vein (71% sensitivity, 86% specificity) for the diagnosis of CCSK were 1.97 and 0.76, respectively.ConclusionPerinephric vessel engorgement and greater tumor enhancement (Tmax/muscle > 1.97 or Tmax/vein > 0.76) are helpful for differentiating between CCSK and Wilms' tumor in children aged below 10 years.

A rare case of spontaneous perirenal hemorrhage - Wunderlich syndrome

Introduction. Spontaneous perirenal hemorrhage or Wunderlich syndrome represents a rare entity in urological settings. The vast majority of the causes are represented by angiomyolipoma and renal cell carcinoma. In other cases, vascular abnormalities, polycystic kidneys, polyarthritis nodosa, or pyelonephritis could represent the cause of perirenal bleeding. The treatment depends on the clinical parameters at the presentation as well as on the possible presence of renal malignancies. Our goal was to present a rare case of a healthy men who presented the idiopathic Wunderlich syndrome. Case outline. We present a case of a 50-year-old patient with spontaneous perirenal hemorrhage that was not due to any defined cause even after a six-months follow-up. Conclusion. In some rare cases of perirenal bleeding, the cause cannot be defined at the presentation, even with today?s advanced radiological imaging methods. Thus, it is important to be aware of the fact that in those cases, a longer follow-up is needed, knowing that the presence of perirenal hematoma can always obscure the real diagnosis. Sometimes, even in cases where the proper follow-up has been done, the real cause of the bleeding remains unknown.

Expression of Carbonic Anhydrase-IX and Vascular Endothelial Growth Factor in Renal Cell Carcinoma and their Prognostic Significance

Introduction: Renal Cell Carcinoma (RCC) is the most common adult renal malignancy. Histopathologically, clear cell RCC accounts for 65-70% of all RCCs. Carbonic Anhydrase-IX (CA-IX) is a transmembrane protein and takes a role in cancer development and progression. A 75-100% clear cell RCCs show CA-IX expression. But this varies with grade and stage of tumour. Vascular Endothelial Growth Factor (VEGF) is responsible for tumour angiogenesis and expressed variedly in RCCs. Both VEGF and CA-IX expression is mediated by Hypoxia Inducible Factor-1α (HIF-1α). Aim: The present study aimed to evaluate the expression of CA-IX and VEGF in respect to different grades and stages of RCC and assessing their prognostic significance. Materials and Methods: This was a cross-sectional, observational study done on 45 histopathogically diagnosed cases of RCC. It was performed in the Department of Pathology, Nil Ratan Sircar Medical College, Kolkata, West Banglore, India over a period of two years (February 2018 to January 2020). Expression of VEGF and CA-IX were studied by immunohistochemistry. Results were analysed in Statistical Package for Social Sciences (SPSS) software (version 16.0) using Pearson Chi-square test. A p-value of <0.05 was regarded as significant. Results: Out of 45 cases of RCC, 34 tumours (32 clear cell carcinoma and two multilocular cystic renal neoplasm of low malignant potential) were evaluated for CA-IX immuno expression. About 25 cases showed CA-IX positivity which inversely associated with grade and stage of RCC (p-value <0.05). The CA-IX had a diagnostic value in detecting clear cell RCC with sensitivity 73.53%, specificity 100% and accuracy 80%. With 28 (62.2%) cases of RCC showed VEGF positivity among which nine were VEGF 1+ and 19 cases VEGF 2+. The VEGF expression showed a positive association with the grade and Tumour-Node-Metastasis (TNM) stage of tumour (p-value <0.05). Finally, authors found a statistically significant inverse association between CA-IX and VEGF expression in RCCs with clear cell morphology including clear cell RCC and multilocular cystic renal neoplasm of low malignant potential (p-value=0.001). Conclusion: High grade RCCs show low expression of CA-IX and strong positivity with VEGF. Both these markers have a prognostic significance. From the therapeutic point of view, VEGF positive tumours, especially inoperable and metastatic cases, may be benefited by anti-VEGF therapy whereas CA-IX positive tumours respond well by treatment with Interleukin-2.

Современные методы лучевой диагностики новообразований почки (обзор литературы)

We presented an overview of domestic and foreign studies on the diagnosis of renal malignancies published in publicly available electronic specialized medical publications. Taking into account that every year the share of oncological diseases in the structure of the total incidence is constantly growing, and that cancer is one of the main causes of death and disability in the working age population, currently, the search for new diagnostic methods to detect kidney tumors still remains a pressing problem located at the junction of several medical disciplines, in particular, oncology, urology, radiation diagnostics and radiation therapy. Over the past decade, the diagnosis of malignant kidney neoplasms has undergone significant changes and has stepped far forward. Because of that the ability to detect the disease in the early stages of development increases. In the study, we examined the most widespread methods, methods that have already lost relevance, as well as new methods, such as magnetic resonance imaging, computed tomography ultrasonography, radiography, etc. We also considered the possibilities of differential diagnosis of benign and malignant neoplasms.

MicroRNA-215-5p Inhibits the Proliferation and Migration of Wilm's Tumor Cells by Targeting CRK.

Objective:Wilm’s tumor is a common renal malignancy in childhood with unsatisfactory prognosis. microRNA-215-5p (miR-215-5p) has been reported as a tumor-suppressive miRNA in different types of human cancers, but rarely in the Wilm’s tumor. In light of this, we tried to investigate the regulatory role and underlying mechanism of miR-215-5p in the Wilm’s tumor.Methods:After sample collection and cell culture, the expression of miR-215-5p and CT10 Regulator of Kinase (CRK) was detected. Then rhabdoid tumor cell lines (formerly classified as Wilms’ tumor cell lines), G401 and WT-CLS1 cells were transfected with pcDNA3.1, pcDNA3.1-CRK, sh-NC, sh-CRK, agomir NC, miR-215-5p agomir, antagomir NC or miR-215-5p antagomir to explore the function of miR-215-5p and CRK in the Wilm’s tumor cell proliferation and migration. Moreover, the relationship between miR-215-5p and CRK was analyzed by dual luciferase reporter gene assay.Results:Lowly-expressed miR-215-5p and highly-expressed CRK were observed in the Wilm’s tumor tissues and cells. Transfection of pcDNA3.1-CRK or miR-215-5p antagomir could promote G401 and WT-CLS1 cell proliferation and enhance migration ability, while transfection of sh-CRK or miR-215-5p agomir led to opposite results. Additionally, miR-215-5p may bind to CRK. Moreover, transfection of pcDNA3.1-CRK in G401 and WT-CLS1 cells could partially reverse the inhibitory effect of miR-215-5p agomir on the proliferation and migration of Wilm’s tumor cells.Conclusion:Our study highlighted that miR-215-5p could suppress the proliferation and migration of Wilm’s tumor cells by regulating the expression of CRK, providing new ideas for molecular targeted therapy for Wilm’s tumor.

Prevalence and Determinants of Atrial Fibrillation-Associated In-Hospital Ischemic Stroke in Patients With Acute Myocardial Infarction Undergoing Percutaneous Coronary Intervention

Atrial fibrillation (AF) is an established risk factor ischemic stroke (IS) and is commonly encountered in patient hospitalized with acute myocardial infarction (AMI). Uncommonly, IS can occur as a complication resulting from percutaneous coronary intervention (PCI). There is limited real world data regarding AF-associated in-hospital IS (IH-IS) in patients admitted with AMI undergoing PCI. We queried the National Inpatient Sample database from January 2010 to December 2014 to identify patients admitted with AMI who underwent PCI. In this cohort, we determined the prevalence of AF associated IH-IS and compared risk factors for IH-IS between patients with AF and without AF using multivariable logistic regression models. IH-IS was present in 0.46% (n = 5,938) of the patients with AMI undergoing PCI (n = 1,282,829). Prevalence of IH-IS in patients with AF was higher compared with patients without AF (1.05% vs 0.4%; adjusted odds ratio: 1.634, 95% confidence interval: 1.527 to 1.748, p <0.001). Regardless of AF status, prevalence and risk of IH-IS was higher in females and increased with advancing age. There was significant overlap among risk-factors associated with increased risk of IH-IS in AF and non-AF cohorts, except for obesity in AF patients (adjusted odds ratio: 1.268, 95% confidence interval: 1.023 to 1.572, p = 0.03) in contrast to renal disease, malignancy, and peripheral vascular disease in non-AF patients. In conclusion, IH-IS is a rare complication affecting patients undergoing PCI for AMI and is more likely to occur in AF patients, females, and older adults, with heterogeneity among risk factors in patients with and without AF.

Clinico-radiological Profile of Posterior Reversible Encephalopathy Syndrome and Its Associated Risk Factors in PICU: A Single-center Experience from a Tertiary Care Hospital in Bhubaneswar, Odisha.

ObjectivePosterior reversible encephalopathy syndrome (PRES) is a clinico-radiographic entity of heterogeneous etiologies having similar clinical and neuroimaging features. Pediatric data are sparse, making early diagnosis challenging, which needs a high index of suspicion. So, we conducted this study to evaluate clinico-radiological features, associated risk factors, etiology, and outcome in children.Materials and methodsThis is a retrospective case series of patients, diagnosed as having PRES and followed up at a tertiary care hospital in Eastern India between September 2016 and December 2019.ResultsAmong 16 patients with a median age of 9.5 years [interquartile range (IQR) 8–13.75] and a male preponderance (75%), common underlying diseases were post-streptococcal glomerulonephritis (56.3%) and renovascular hypertension (12.5%). Acute elevation of blood pressure was found in all patients (n = 16). The neurological symptom was seizure (87.5%), mental changes (68.75%), headache (43.8%), vomiting (31.3%), and visual disturbances (31.3%). The most common triggering factor was hypertension (100%), use of mycophenolate mofetil and prednisolone (12.5%), and hemodialysis (12.5%). Anemia was present in 15 (93.4%) patients at the time of admission. All showed abnormal neuroimaging with 55% having atypical involvement. The most common site was the parietal-occipital cortex (88%), frontal and temporal lobe (44% cases each), and the cerebellum (13%). Clinical recovery was followed by a radiological resolution in all survived except in one, who developed visual impairment.ConclusionPosterior reversible encephalopathy syndrome should be considered in the differential diagnosis of patients who present with acute neurological disturbances and underlying diseases such as renal disorders, vasculitis, malignancy, and use of immunosuppressant accompanied by hypertension. Early diagnosis and treatment of comorbid conditions are of paramount importance for the early reversal of the syndrome.How to cite this articleBehera CK, Jain MK, Mishra R, Jena PK, Dash SK, Sahoo RK. Clinico-radiological Profile of Posterior Reversible Encephalopathy Syndrome and Its Associated Risk Factors in PICU: A Single-center Experience from a Tertiary Care Hospital in Bhubaneswar, Odisha. Indian J Crit Care Med 2020;24(12):1223–1229.

Current Recommendations, Controversies, and Potential Novel Approaches in the Treatment of Wilms Tumor

Wilms tumor (WT) is one of the most common renal malignancies in children, comprising about 5% of all childhood cancers. If diagnosed early, WT responds well to appropriate interventions such as surgical resection, chemotherapy and radiotherapy, with a 5 year survival higher than 85%. In this review, we will describe the first-line treatment options for WT, discuss controversies related to particular modes of therapy, and highlight promising advances in molecular biology that may serve as effective therapeutics in the near future. Current treatment protocols for WT include nephrectomy followed by postoperative chemotherapy with or without preoperative chemotherapy. Though both are acceptable forms of treatment, preoperative chemotherapy prior to tumor resection has been shown to reduce tumor size and decrease the risk of intraoperative tumor rupture. Preoperative transcatheter arterial chemoembolization has also been shown to improve tumor resections and relapse-free survival rates, potentially providing an additive method to improve WT outcomes. Radical nephrectomy is the mainstay surgical treatment for WT, however evidence suggests that partial nephrectomies may be an equally viable option. Radiotherapy traditionally utilizes the anteroposterior-posteroanterior field technique, but recent advances have allowed for tumor-specific targeting and sparing of non-neoplastic tissues using intensity-modulated radiation therapy and volumetric-modulated arc therapy. Lastly, potential targets for future therapy include the β-catenin pathway, which has been found to be important in the development of WT, in addition to advances in applying microRNA, M6620, and stem cell therapy.

Wilms Tumor in Sub-Saharan Africa: Molecular and Social Determinants of a Global Pediatric Health Disparity.

Wilms tumor (WT) is the most common renal malignancy of childhood. Global disparities in WT have been reported with the highest incidence and lowest overall survival occurring in sub-Saharan African nations. After a detailed search of PubMed, we reviewed available literature on WT in sub-Saharan Africa and summarized findings that explore biologic and social factors contributing to this alarming cancer health disparity. Access to care and treatment abandonment are the most frequently reported factors associated with decreased outcomes. Implementation of multidisciplinary teams, collaborative networks, and financial support has improved overall survival in some nations. However, treatment abandonment remains a challenge. In high-income countries globally, WT therapy now is risk-stratified according to biology and histology. To a significantly lesser extent, biologic features have been studied only recently in sub-Saharan African WT, yet unique molecular and genetic signatures, including congenital anomaly-associated syndromes and biomarkers associated with treatment-resistance and poor prognosis have been identified. Together, challenges with access to and delivery of health care in addition to adverse biologic features likely contribute to increased burden of disease in sub-Saharan African children having WT. Publications on biologic features of WT that inform treatment stratification and personalized therapy in resource-limited regions of sub-Saharan Africa have lagged in comparison to publications that discuss social determinants of health. Further efforts to understand both WT biology and social factors relevant to appropriate treatment delivery should be prioritized in order to reduce health disparities for children residing in resource-limited areas of sub-Saharan Africa battling this lethal childhood cancer.

Patient and graft outcome among Filipinos with post-kidney transplant malignancy

Background: Improvements in the care of kidney transplant recipients (KTRs) resulted to longer life expectancy and increased survival rate. However, studies showed an increase in the incidence of post-kidney transplant malignancy (PTM) affecting the survival and quality of life of the said patients. Methods: This is a retrospective cross-sectional study among 91 patients in a tertiary referral center. The primary endpoints were patient and graft survival at 12 and 24 months after diagnosis and/or treatment of malignancy. Kaplan-Meier analysis was applied to estimate probabilities of survival. Results: The overall incidence of patients developing malignancy after kidney transplant was 1.4% with patient median age at 57 years on diagnosis. The median period from kidney transplant to malignancy diagnosis was at 7.39±5.94 years. The most com mon primary sites of malignancy seen were colorectal, breast, and renal malignancies. The overall graft survival probability of PTM patients was 98.6% and 93.6% at 12 and 24 months, while the overall patient survival probability of PTM patients was 75.5%, 63.3%, and 48.8% at 12, 24, and 64 months, respectively. Conclusions: The incidence of PTM in our study was lower compared with other countries. The overall patient survival rate, how ever, was much lower than those who did not develop PTM. Moreover, our study showed lower patient survival rate compared with other countries. Routine screening for cancers among our KTRs following our local guidelines should be adhered to. The type of cancer among Filipino KTRs were also different from what were reported in other publications. Further studies that will look into risk factors to developing malignancy after KT among Filipinos and the role of type and dose of immunosuppressant is recommended.

A rare case of right-sided varicocele in right renal tumor in the absence of venous thrombosis and IVC compression

BackgroundThe presence of unilateral right-sided varicocele hints at a serious retroperitoneal disease such as renal cell neoplasm. Such tumors are usually associated with a thrombus in renal vein or spermatic vein. We report a rare presentation of right-sided renal tumor causing right-sided varicocele in the absence of thrombus in renal vein and spermatic vein but due to an anomalous vein draining from the tumor into the spermatic vein as demonstrated by computed tomography angiogram.Case presentationA 54-yr-old hypertensive male presented with unilateral grade 3 right-sided varicocele and no other signs and symptoms. Ultrasound examination of his abdomen showed the presence of a mass lesion in the lower pole of right kidney. Computed tomography confirmed the presence of right renal mass, absence of thrombus in right renal vein or inferior vena cava. The angiographic phase of CT scan showed an anomalous vein from the tumor draining into the pampiniform plexus causing varicocele.ConclusionThe presence of right-sided varicocele should raise a suspicion hidden serious pathological retroperitoneal condition, renal malignancy in particular, and should prompt the treating physician to carry out imaging studies of the retroperitoneum and careful study of the angiographic phase of the CT scan can ascertain the pathogenesis of the varicocele.