Clinical study of steroid receptors in nonmuscle invasive bladder cancer: A domain worth revisiting.

Introduction: The prognostic significance of steroid receptors in bladder cancer remains controversial. This study was designed to determine the expression status of androgen receptor (AR), estrogen receptors (ERα and Erß), and its potential role in predicting survival in patients with nonmuscle invasive bladder cancer (NMIBC). Methods: Sixty patients of NMIBC were screened and 57 (41 males and 16 females) were included in our study. The tissue microarray slides were evaluated by pathologists blinded to the clinical information. Association of distribution of steroid receptors with stage, grade, progression, and recurrence was seen. Results: The mean age of the population was 60.9 ± 9.3 years. Pathologically, majority of the patients were Ta (Ta: T1 stage 61.4% vs. 38.6%). Nine (15.8%) of the tumors stained positive for AR while one (1.8%) tumor stained positive for ERα and 36 (63.2%) tumors stained for ERß. A higher proportion of male NMIBC stained positive for AR (19.5% vs. 6.2%, P = 0.420) while ERß positivity was higher in females (58.5% vs. and 75%,P = 0.247). AR-negative tumors showed higher recurrence (20/48%-42%) as compared to AR-positive tumors (2/9%-22%). ERß-positive tumors showed higher recurrence (15/36%-42% vs. 7/21%-33%, P = 0.179). Progression-free survival (PFS) was found to be significantly lower for ERß-negative group (log-rank test P = 0.035). Conclusion: AR and ERß positivity is found in NMIBC patients while ERα shows minimal staining in NMIBC patients. Although it did not reach a statistical significance, a higher proportion of AR-negative and ERß-positive tumors recurred as compared to AR-positive and ERß-negative patients. PFS was significantly lower in ERß-negative group. Further exploratory studies on larger sample sizes are required to validate these findings in NMIBC patients.

Accuracy of combined multi-parametric MRI and PSMA PET-CT in diagnosing localized prostate cancer: newer horizons for a biopsy-free pathway.

INTRODUCTION: Prostate-specific antigen (PSA) is a reliable biomarker for identification of prostate cancer, although a biopsy is still the gold standard for detecting prostate cancer. Similar to higher PIRADS lesions on MRI, the maximal standard uptake value (SUV max) on PSMA PET is linked to a higher likelihood of prostate cancer. Can an mpMRI in conjunction with PSMA PET Scan accurately predict prostate cancer and further trigger omission of biopsy similar to other solid organ urological malignancies? METHODS: Ga-68 PSMA PET and mpMRI were performed for each patient who was a part of this retrospective study. The PET-positive lesion's maximum standardized uptake value (SUVmax) was recorded. Prostate biopsies were performed on patients who had PSMA PET avid lesions and a PIRADS score of 4 or 5. Robot-assisted radical prostatectomy (RARP) was afterward performed on patients who had cancer on their prostate biopsy. The prostatectomy specimen's histopathological information was recorded. Cutoff values and correlations between the variables were determined using the ROC curves and Pearson's correlation test. RESULT: On the basis of suspicious DRE findings or elevated PSA, 70 men underwent mpMRI and PET scans. PIRADS 4 patients had a median (IQR) SUVmax of 8.75 (11.95); whereas, PIRADS 5 patients had an SUVmax of 24.5 (22). The mean SUVmax for patients whose biopsies revealed no cancer was 6.25 ± 1.41. With an AUC of 0.876 on the ROC curve, it was found that there was a significant positive correlation between the results of the mpMRI and PET scans and those of the histopathological investigation. A SUVmax ≥ 8.25 on PSMA PET for a PIRADS 4/5 lesion on mpMRI will aid in correctly predicting malignancy, with a sensitivity of 82.8% and specificity of 100%. CONCLUSION: The findings of this study were positive and indicated that patients with a high suspicion of prostate cancer on mpMRI and PSMA PET (PIRADS ≥ 4 and SUVmax ≥ 8.25). This study substantiates the fact that a combination of mpMRI and PSMA PET can accurately predict localized prostate cancer.

Oligometastatic carcinoma prostate - An overview of the last decade.

Introduction: Oligometastatic prostate cancer (OMPC) has gained profound interest lately due to its different tumor biology and our ability to use multimodality therapy for cure or prolonged survival. Selecting the appropriate patient for treatment has become the aim of treating urologists, medical oncologists, and radiation oncologists. Through this review, we try to highlight the management of OMPC in light of recent literature. Methods: Literature search was performed on Pubmed, Scopus and Embase using keywords "Oligometastatic", " Prostate Cancer" using operators such as "And" & "Or". Relevant articles were screened and all the latest articles on this emerging entity were included in this review. Results: All trials relevant to oligometastatic prostate cancer defining the role of surgery, radiotherapy and systemic therapy were included and appropriate inferences were drawn. Relevant studies were compiled in tabular form for this article. Conclusion: The current standard of care of management for OMPC remains systemic therapy on the lines of hormone-sensitive metastatic prostate cancer. The evolving role of surgery, and radiotherapy along with systemic therapy is highlighted in this article.

Preoperative Predictors for the Presence of Motile Spermatozoa in the Epididymis and Patency of Anastomosis during Microsurgical Vasoepididymal Anastomosis in Patients with Obstructive Azoospermia.

Background: Following microsurgical vaso-epididymal anastomosis (VEA), anastomotic patency with sperm returning to the ejaculate is not always present and may even be delayed. The presence of motile spermatozoa is highly suggestive of future patency following surgery. Aims: We prospectively analyse the factors that could predict motile spermatozoa at the epididymis intraoperatively and predictors of patency in patients with obstructive azoospermia (OA) undergoing microsurgical VEA. Settings and Design: Department of Urology of a tertiary care centre in Northern India. It is a prospective observational study. Materials and Methods: Over a 2-year period (July 2019 to June 2021), 26 patients with idiopathic OA were enrolled in the study. Twenty patients underwent microsurgical VEA. Patients were divided into two groups based on the presence/absence of intraoperative motile spermatozoa. Statistical Analysis Used: Analysis of preoperative and intraoperative factors was done using the Mann-Whitney U-test, Chi-squared test and Fischer exact test. Results: Out of 20 patients, 5 (group 2) had intraoperative motile spermatozoa in the epididymal fluid and 15 (group 1) had nonmotile spermatozoa. Low luteinising hormone (LH) levels (P = 0.01) and high testosterone levels (P = 0.05) were the predictive of presence of motile spermatozoa in epididymal fluid. Mean follow-up was 9 months (6-18 months). Predictors of higher patency were grade 2 epididymis (firm, turgid and tense) (P = 0.003), low LH levels (P = 0.03), low sertoli cell index (P = 0.006), high sperm-Sertoli index (P = 0.002) and better surgeon satisfaction (P = 0.01). Conclusion: Low LH levels and high testosterone levels may be predictive of the presence of motile spermatozoa in epididymal fluid. Firm, turgid and tense epididymis, low Sertoli cell index, high sperm-Sertoli index and surgeon satisfaction suggest a greater chance of success after VEA for idiopathic azoospermia.

Iatrogenic partial glanular amputation: A rare complication of circumcision.

Circumcision is a customary ritual across many cultures. However, the safety of such procedures remains a concern. A boy underwent circumcision in 2014 by a religious worker at the age of 7 years. Post circumcision, the patient had gradual narrowing of the penis, between the glans and the shaft, with an iatrogenic partial glanular amputation and presented to us at the age of 14 years. The patient underwent end-to-end urethroplasty and glansplasty. Postoperatively, the patient did well and the wound remained healthy. Circumcision has complications even in expert hands. Religious circumcision can result in dreadful complications in children and adolescents.

Comparison of efficacy of three commonly used alpha-blockers as medical expulsive therapy for distal ureter stones: A systematic review and network meta-analysis

ABSTRACT Introduction: The efficacy of alpha-blockers as medical expulsive therapy (MET) is well established. However, it is not known which of the three most commonly used alpha-blockers (tamsulosin, alfuzosin and silodosin) is the most efficacious. With this study we aimed to assess the efficacy of the three commonly used alpha-blockers as MET for distal ureter stones. Materials and Methods: For this review, we searched multiple databases such as PubMed/Medline, Scopus, Embase, OviD SP, CINAHL, and web of science to identify all the relevant randomized studies comparing the efficacy of tamsulosin, alfuzosin, and silodosin. Preferred reporting items for systematic reviews for network meta-analysis (PRISMA-NMA) were followed while conducting this review and the study protocol was registered with PROSPERO (CRD42020175706). Results: In this review, 31 studies with 7077 patients were included. Compared to placebo all the treatment groups were more effective for both stone expulsion rate (SER) and stone expulsion time (SET). For both SER and SET, silodosin had the highest SUCRA (94.8 and 90.4) values followed by alfuzosin (58.8 and 64.9) and tamsulosin (46.2 and 44.5). The incidence of postural hypotension was similar with all the drugs, whereas, the incidence of retrograde ejaculation was significantly higher for silodosin. Overall confidence for each comparison group in this review ranged from "very low" to "moderate" according to the CINeMA approach. Conclusion: Among the three commonly used alpha-blockers silodosin is the most efficacious drug as MET for lower ureter stones followed by alfuzosin and tamsulosin.

What should urologists know about Pseudojournals and open access publishing? A narrative review of the literature.

Introduction: The majority of the open access publishing allows the researchers to publish their articles for a fee and at the same time enables the readers to access the research without paying the expensive journal subscription charges. Under the garb of open access publishing, predatory journals run a scam to dupe the researchers of money. This study was conducted to highlight the characteristics of pseudojournals and increase the awareness about their modus operandi. Methods: The email inboxes of 3 academic urologists (APS, AS, and KP) were searched for emails soliciting articles for open access journals. A list of all such journals was compiled. These journals were checked for metrics from the Journal Citation Reports and the Scimago Journal Rankings. All these journals were then cross-checked with the available whitelists and blacklists. Features pointing toward a pseudo journal were identified as red flag signs for these journals and were noted. A literature search was performed on open access publishing and predatory journals, and the salient points were noted. A checklist of red flag signs was compiled. Results: A total of 71 emails soliciting article submissions from 68 journals were received by the three urologists (APS, AS, KP). Of these, 54 were highly suggestive of being a pseudojournal, 5 journals were operating in the gray zone between genuine open access journals and outright predatory journals, and 9 were genuine open access journals. A total of 33 articles on predatory journals were reviewed after the literature search as per the PRISMA guidelines. The red flag signs identified along with the literature review were used to create the SAFEiMAP checklist, which can be used to identify predatory journals. Conclusion: Predatory journals have infiltrated the whitelists, and the indexing databases like PubMed and no blacklist is all-inclusive. Understanding the concept and the types of open access publishing gives the researchers a better idea on how to differentiate fake journals from the genuine ones. Using a checklist will help to identify the red flag signs of such journals and identify those journals that operate in the gray zone.

Robot-assisted partial nephrectomy for moderate to highly complex renal masses. A systematic review and meta-analysis.

Introduction: Multiple studies have been published recently assessing feasibility of robot-assisted partial nephrectomy (RAPN) for moderate to highly complex renal masses. Some studies have even compared partial nephrectomy (PN) performed through various modalities such as open PN (OPN) versus RAPN and laparoscopic PN (LPN) versus OPN. The primary aim of this review was to analyze perioperative outcomes such as warm ischemia time (WIT), duration of surgery, estimated blood loss (EBL), complications, blood transfusion, length of stay, and margin status following RAPN for complex renal masses. Another objective was to compare perioperative outcomes following various surgical modalities, i.e., OPN, LPN, or RAPN. Methods: Literature search was conducted to identify studies reporting perioperative outcomes following RAPN for moderate (Radius, Endophytic/Exophytic, Nearness, Anterior/posterior location [RENAL] score 7-9 or Preoperative Aspects of Dimension used for anatomic classification [PADUA] score 8-9) to high complexity renal masses (RENAL or PADUA score ≥ 10). Meta-analysis of robotic versus OPN and robotic versus LPN was also performed. Study protocol was registered with PROPSERO (CRD42019121259). Results: In this review, 22 studies including 2,659 patients were included. Mean duration of surgery, WIT, and EBL was 132.5-250.8 min, 15.5-30 min, and 100-321 ml, respectively. From pooled analysis, positive surgical margin, need for blood transfusion, minor and major complications were seen in 3.9%, 5.2%, 19.3%, and 6.3% of the patients. No significant difference was noted between RAPN and LPN for any of the perioperative outcomes. Compared to OPN, RAPN had significantly lower EBL, complications rate, and need for transfusion. Conclusions: RAPN for moderate to high complexity renal masses is associated with acceptable perioperative outcomes. LPN and RAPN were equal in terms of perioperative outcomes for complex masses whereas, OPN had significantly higher blood loss, complications rate, and need for transfusion as compared to RAPN.

Quality of Life, Efficacy, and Safety of Sequential Intravesical Gemcitabine + Docetaxel versus BCG for Non-Muscle Invasive Urinary Bladder Cancer: A Pilot Study.

PURPOSE: Bacille Calmette-Guerin (BCG) is considered the most effective agent for non-muscle invasive bladder cancer (NMIBC). However, due to BCG-related toxicity, multiple studies have suggested the role of newer chemotherapeutic drugs. The aim of our study was to evaluate intravesical gemcitabine + docetaxel (Gem/Doce) versus BCG with respect to quality of life (QOL), safety, and efficacy in NMIBC. METHODS: A total of 60 patients with NMIBC were evaluated between July 2019 and December 2020 in a prospective manner. The sample size calculation was done, keeping in mind the incidence of intravesical BCG-related complications of up to 50-60% and 20-30% for Gem/Doce combination. The p value of 0.05 was kept as statistically significant. The enrollment ratio was kept at 1, and power of study was aimed at 80%. The study population was alternatively assigned to two groups (BCG vs. Gem/Doce) of 30 patients each. Both the groups received 6 weekly doses of induction therapy followed by 6 monthly doses of maintenance therapy if no recurrence was noted at interim follow-up. QOL scores, safety, and efficacy were assessed at beginning of intravesical therapy, end of induction, and 6 months of maintenance therapy. Cystoscopy examination and cytology were performed at the end of induction therapy and 3-monthly thereafter. RESULT: The preliminary results at the end of 6 months following maintenance therapy showed that the demographic profile, histological stage, and grade were comparable between two groups. The QOL scores using QLQ-30 and QLQ-BLS-24 showed statistically significant differences with the Gem/Doce arm showing better outcomes. There were no progressions to higher stage, while one recurrence each was seen in both groups. Patient-related side effects measured by CTCAE (Common Terminology Criteria for Adverse Events)version 5 showed that the BCG group had higher toxicity profile as compared to Gem/Doce group. CONCLUSION: Gem/Doce combination intravesical therapy is a promising alternative to BCG for treatment of NMIBC, showing better QOL measures and lesser side effects.

Primary bladder neck obstruction in female: 'an enigmatic disorder'.

A 36-year-old woman presented with problems of lower urinary tract symptoms for 2 years duration. Patient was being managed as a case of urethral stricture with routine calibration. Micturating cystourethrogram showed failure of bladder neck to open. On urodynamic study, she was found to have bladder outlet obstruction with high pressure, low flow pattern. Based on these findings, patient was diagnosed to have primary bladder neck obstruction (PBNO). She was also being evaluated for primary infertility and was to undergo in vitro fertilisation. She successfully underwent bladder neck incision after discussion about management options. Bladder neck incision is one of the accepted management options for PBNO. Post procedure patient was relieved of symptoms and also had an uneventful full-term pregnancy. Bladder neck incision in women is an effective treatment option when patient has been properly selected and procedure done with expert hands.

Long-term impact of testicular torsion and its salvage on semen parameters and gonadal function.

Introduction: Testicular torsion is a urological emergency, and long-term outcomes of testicular torsion on infertility, hormonal function, and salvaged testicular size are unclear. Materials and Methods: We conducted an ambispective, observational study from January 2014 to December 2019. Baseline demographics, time of presentation, clinical features, and management details of all the patients of testicular torsion were recorded from the database. All the patients were followed up in the outpatient clinic for testicular size, hormone levels, semen analysis, and erectile function. Results: Of 85 patients, only 67 could be contacted and included in the final analysis. Group 1(orchiectomy) comprised 44 patients, and Group 2(salvage) had 23 patients. Follow-up duration ranged from 2 to 6 years and mean follow-up was 42 ± 12 months. The median time to presentation was significantly higher in Group 1 (48 hours) as compared to Group 2 (12 hours). The rate of testicular salvage did not vary with age of the patients. Doppler ultrasonography of the scrotum detected 92.5% of all cases of torsion. Antisperm antibody levels were within normal range in all patients. Approximately 47% of patients in the salvage group developed testicular atrophy on follow-up. Serum testosterone level was significantly lower in Group 1 and the subset of patients with testicular atrophy. Rest of the hormonal parameters, semen analysis, and erectile function were comparable between two groups. Conclusion: The time between onset and presentation is an important contributing factor in guiding testicular salvage. Even after salvage, many testes may atrophy on follow-up. Orchiectomy and testicular atrophy in the long term have negative impact on serum testosterone. The patients should be counseled for a long-term follow-up for the risk of testicular atrophy and low testosterone levels.

Utility of WhatsApp in emergency urological practice: An interrater reliability study.

INTRODUCTION: The messaging application 'WhatsApp' is used in clinical practice, often for communication between a medical trainee and a consultant. We designed this study to find the interrater reliability of the data transmitted through this application and validating its use in urological practice. MATERIALS AND METHODS: Clinical details and computerized tomographic (CT) images of 30 patients visiting the urology emergency were posted in a closed WhatsApp group involving three consultants (SKD, APS, and KC). The CT images were posted in the WhatsApp group as Whole Image (WI) and Image of Interest (IOI) format and rated on a scale of 1-5. The consultants formulated a provisional diagnosis and initial management strategy. The interrater reliability of these responses was analyzed in the study. RESULTS: Mean WI rating ranged from 3.03 ± 0.61 to 3.73 ± 0.64 (Cronbach alfa [α]-0.494, P = 0.006). Mean IOI rating ranged from 3.4 ± 0.56 to 4.13 ± 0.73 (α-0.824, P < 0.0001). For diagnosis, the proportion of observed agreement (P0) was 83.3% for SKD and APS, 76.6% for SKD and KC, and 73.3% for APS and KC. For management, P0 was 86.6% for APS and KC, 86.6% for SKD and APS, and 80% for SKD and KC. CONCLUSIONS: WhatsApp Messenger serves to transmit good quality pictures of CT scan images. A reasonable diagnosis and management strategy can be formulated using this app with fair inter-rater reliability.

'Pee'BuSt Trial: A single-centre prospective randomized study comparing functional and anatomic outcomes after augmentation urethroplasty with penile skin graft versus buccal mucosa graft for anterior urethral stricture disease.

PURPOSE: To assess the surgical outcomes of augmentation urethroplasty (AU) using penile skin graft (PSG) compared to buccal mucosa graft (BMG) in anterior urethral stricture disease. METHOD: Between January 2018 and January 2019, 100 patients with anterior urethral stricture planned for AU were randomized into PSG or BMG arms (CTRI/2018/07/015028). Anatomic and functional variables were compared pre-operatively and post-operatively. Primary outcome was success rate at 18 months and it was defined if any of the three criteria were met, i.e. either maximum urinary flow (Qmax) > 15 ml/s or urethral calibration of 16 French or ability to traverse the repair with 17 French cystoscope. Secondary outcomes were functional parameters such as International Prostate Symptom Score (IPSS), International Index of Erectile Function (IIEF) Score, Male Sexual Health Questionnaire for Ejaculatory Dysfunction (MSHQ-EJD), and Urethral Stricture Surgery-Patient Related Outcome Measure (USS-PROM). RESULTS: Pre-operative variables were comparable between both the arms. Median duration of follow-up was 22 months (18-24 months). At 18 months, the success rates of AU with PSG and BMG were comparable (89% v/s 91%; p = 0.70, 95% CI-0.33 to 5.21). The improvements in Qmax (p = 0.06), IPSS (p = 0.43) and USS-PROM (p = 0.49) were comparable between the two arms. There was no statistically significant difference in the IIEF-Erectile domain (p = 0.07), IIEF-Orgasmic domain (p = 0.11) and MSHQ-EJD (p = 0.20) following AU at 18 months. Clavien-Dindo grade I complications were 12.7% in PSG and 16.7% in BMG. CONCLUSION: This study provides level 1 evidence of no statistical significant difference in outcomes of AU using BMG or PSG.

Atrophic Kidney-Like Lesion - Case Report of A Provisional Entity with Brief Review of Literature.

Atrophic kidney-like lesion is a recently recognized entity, post 2016 World Health Organization Classification of tumors of the urinary system. The behavior of this tumor is not fully known as only a handful of cases with limited follow-up are available. This entity closely mimics thyroid-like follicular carcinoma of the kidney, which has different prognosis. We report a case of incidentally detected atrophic kidney-like lesion in an elderly gentleman who had urothelial carcinoma of the urinary bladder with a brief review of literature. Atrophic kidney-like lesion and urothelial carcinoma of the urinary bladder association has not been reported in the literature.

Comparison of efficacy of three commonly used alpha-blockers as medical expulsive therapy for distal ureter stones: A systematic review and network meta-analysis.

INTRODUCTION: The efficacy of alpha-blockers as medical expulsive therapy (MET) is well established. However, it is not known which of the three most commonly used alpha-blockers (tamsulosin, alfuzosin and silodosin) is the most efficacious. With this study we aimed to assess the efficacy of the three commonly used alpha-blockers as MET for distal ureter stones. MATERIALS AND METHODS: For this review, we searched multiple databases such as PubMed/Medline, Scopus, Embase, OviD SP, CINAHL, and web of science to identify all the relevant randomized studies comparing the efficacy of tamsulosin, alfuzosin, and silodosin. Preferred reporting items for systematic reviews for network meta-analysis (PRISMA-NMA) were followed while conducting this review and the study protocol was registered with PROSPERO (CRD42020175706). RESULTS: In this review, 31 studies with 7077 patients were included. Compared to placebo all the treatment groups were more effective for both stone expulsion rate (SER) and stone expulsion time (SET). For both SER and SET, silodosin had the highest SUCRA (94.8 and 90.4) values followed by alfuzosin (58.8 and 64.9) and tamsulosin (46.2 and 44.5). The incidence of postural hypotension was similar with all the drugs, whereas, the incidence of retrograde ejaculation was significantly higher for silodosin. Overall confidence for each comparison group in this review ranged from "very low" to "moderate" according to the CINeMA approach. CONCLUSION: Among the three commonly used alpha-blockers silodosin is the most efficacious drug as MET for lower ureter stones followed by alfuzosin and tamsulosin.

Trifecta and pentafecta outcomes following robot-assisted partial nephrectomy for hilar versus nonhilar tumors: A propensity-matched analysis.

INTRODUCTION: Hilar tumors are a unique subset of complex renal masses posing a potential surgical challenge during partial nephrectomy. The outcomes of hilar masses have not been compared to non-hilar renal masses of similar RENAL nephrometry score (RNS). In this study, we analyzed the outcomes of hilar versus nonhilar masses after a propensity score matching. METHODS: Prospectively maintained database of patients who underwent robot assisted PN between November 2014 and December 2018 was abstracted for hilar and nonhilar tumors. We performed propensity matching for baseline variables such as age, sex, body mass index, comorbidities, preoperative glomerular filtration rate, and RNS for each patient on the basis of propensity scores. RESULTS: We included 48 patients with hilar tumors and 153 with nonhilar tumors. On propensity matching, 41 patients were included in each group. The mean operative time (162.4 ± 48.9 min vs. 144.1 ± 38.8 min, P = 0.48), warm ischemia time (29.0 ± 8.8 min vs. 24.4 ± 8.2 min, P = 0.12), and the estimated blood loss (201.8 ± 184.7 ml vs. 150.6 ± 160.5 ml, P = 0.37) were not significantly different between the hilar and the nonhilar groups. Trifecta was achieved in only 14/41 (34.1%) of the patients in the hilar group as compared to 24/41 (58.5%) in the nonhilar group (P = 0.027). Logistic regression analysis identified that hilar location of the tumors was not an independent predictor of overall complications (OR 6.37, confidence interval [CI] 0.5-69.4, P = 0.4), trifecta (OR 0.38, CI 0.14-1.0, P = 0.051), and pentafecta outcomes (OR 0.4, CI 0.1-1.51, P = 0.17). CONCLUSIONS: Hilar location was associated with poorer trifecta outcomes compared to the nonhilar tumors. However, hilar location per se was not an independent predictor of overall complications and trifecta and pentafecta outcomes.

Primary primitive neuroectodermal tumour of the kidney in adults: Experience of managing 12 cases with systematic review and pooled analysis of literature.

INTRODUCTION: Primitive neuroectodermal tumor (PNET) of the kidney is unusual in adults. These tumours are diagnosed mainly on histopathology and that too sometimes has limitations. With this study, we aimed to review our clinical and histopathological data of patients with renal PNET and reviewing the world literature. METHODS: In this retrospective study, we reviewed our database from January 2006 to July 2018 to include all the cases of primary PNET of the kidney. We also performed systematic literature search to identify all the relevant series on renal PNET. RESULTS: A total of 12 patients including 5 men and 7 women were managed during the above mentioned period. Out of these 7 patients, 2 patients had metastasis at diagnosis, one had locally advanced disease, 6 underwent radical nephrectomy, 5 patients received adjuvant chemotherapy (two currently receiving) and only 1 patient received adjuvant radiotherapy (RT). On Immunohistochemistry (IHC), CD99 and FLI1 were positive in all the patients. Median survival was 10 months. In our review 10 studies were included, 38.6% of the patients had metastatic disease and 10.7% had locally advanced disease at diagnosis. Overall mean survival was 33.75 months. CD99 and FLI1 were positive in 94.3% and 78.5%, respectively. CONCLUSION: PNET remains a pathological diagnosis and IHC has important place in diagnosis of PNET. Locally advanced and metastatic disease is common at diagnosis leading to overall poor survival.