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To examine the overall and stage-specific age-adjusted incidence, 5-year survival and mortality rates of bladder cancer (BCa) in the United States, between 1973 and 2009. A total of 148,315 BCa patients were identified in the Surveillance, Epidemiology and End Results database, between years 1973 and 2009. Incidence, mortality, and 5-year cancer-specific survival rates were calculated. Temporal trends were quantified using the estimated annual percentage change (EAPC) and linear regression models. All analyses were stratified according to disease stage, and further examined according to sex, race, and age groups. Incidence rate of BCa increased from 21.0 to 25.5/100,000 person-years between 1973 and 2009. Stage-specific analyses revealed an increase incidence for localized stage: 15.4–20.2 (EAPC: +0.5%, p<0.001) and distant stage: 0.5–0.8 (EAPC: +0.7%, p=0.001). Stage-specific 5-year survival rates increased for all stages, except for distant disease. No significant changes in mortality were recorded among localized (EAPC: −0.2%, p=0.1) and regional stage (EAPC: −0.1%, p=0.5). An increase in mortality rates was observed among distant stage (EAPC: +1.0%, p=0.005). Significant variations in incidence and mortality were recorded when estimates were stratified according to sex, race, and age groups. Albeit statistically significant, virtually all changes in incidence and mortality were minor, and hardly of any clinical importance. Little or no change in BCa cancer control outcomes has been achieved during the study period.

Prune belly syndrome (PBS), also known as Eagle-Barret syndrome, is a rare, multi-system congenital myopathy primarily affecting males. Phenotypically, PBS cases manifest three cardinal pathological features: urinary tract dilation with poorly contractile smooth muscle, wrinkled flaccid ventral abdominal wall with skeletal muscle deficiency, and intra-abdominal undescended testes. Genetically, PBS is poorly understood. After performing whole exome sequencing in PBS patients, we identify one compound heterozygous variant in the PIEZO1 gene. PIEZO1 is a cation-selective channel activated by various mechanical forces and widely expressed throughout the lower urinary tract. Here we conduct an extensive functional analysis of the PIEZO1 PBS variants that reveal loss-of-function characteristics in the pressure-induced normalized open probability (NPo) of the channel, while no change is observed in single-channel currents. Furthermore, Yoda1, a PIEZO1 activator, can rescue the NPo defect of the PBS mutant channels. Thus, PIEZO1 mutations may be causal for PBS and the in vitro cellular pathophysiological phenotype could be rescued by the small molecule, Yoda1. Activation of PIEZO1 might provide a promising means of treating PBS and other related bladder dysfunctional states. PIEZO1 is a mechanosensitive ion channel. Here, authors identify PIEZO1 human mutations in Prune Belly Syndrome. At a single molecule level these mutations exhibit loss-of-function characteristics.

Purpose To evaluate whether anti-inflammatory agents affect outcomes in patients receiving intravesical BCG therapy for high-grade (HG) non-muscle-invasive bladder cancer (NMIBC). Methods We reviewed the records of 203 patients in a prospective database of HG NMIBC from 2006 to 2012 at a single institution. Patients who had muscle-invasive disease ( n  = 32), low-grade pathology ( n  = 4), underwent early cystectomy within 3 months ( n  = 25), had <3 months of follow-up ( n  = 11), or did not receive an induction course of intravesical BCG ( n  = 32) were excluded. Clinicopathologic data were tabulated including demographics, comorbidities, pathologic stage and grades, intravesical therapy, and concomitant use of aspirin, NSAIDs, COX inhibitors, and statins. Multivariate Cox regression analysis explored predictive factors for recurrence, progression (stage progression or progression to cystectomy), cancer-specific survival (CSS), and overall survival (OS). Results Ninety-nine patients with HG NMIBC who received at least one induction course of intravesical BCG were identified, with median follow-up of 31.4 months. There were 20 (20.2 %) deaths, including 6 (6.1 %) patients with bladder cancer-related mortality. 13 % patients experienced tumor progression and 27 % underwent cystectomy following failure of intravesical therapy. Anti-inflammatory use included statins (65 %), aspirin (63 %), or non-aspirin NSAIDs/COX inhibitors (26 %). Anti-inflammatory use was not significantly predictive of recurrence, progression, or mortality outcomes on Cox regression. CIS stage was associated with higher progression, while age, BMI, and Charlson score were independent predictors of overall mortality. Conclusion Despite speculation of inhibitory effects on BCG immunomodulation there was no evidence that anti-inflammatory agents impacted oncologic outcomes in patients receiving BCG for HG NMIBC.

Purpose of Review The treatment of pediatric renal tumors is nuanced and complex. This review highlights these nuances and aims to prepare the surgeon for treating these complex patients. Recent Findings Cooperative group study has improved survival for several pediatric renal tumors significantly, namely, Wilms tumor and clear cell sarcoma. There are several more rare tumors however, which have not achieved the same level of success and still require more study to find the optimal treatment regimen to achieve excellent overall survival. These tumors include rhabdoid tumor and renal medullary carcinoma. Several COG Wilms tumor protocols have recently closed and new ones are set to release soon. It is anticipated that these studies will continue to shift focus to minimizing adverse events/late effects by potentially eliminating or reducing chemotherapeutic exposures while maintaining excellent overall survival. Specifically, new discovery of molecular markers in Wilms tumor will play a significant role with respect to risk stratification. Already, loss of heterozygosity at both 1p and 16q prompts treatment intensification as they are associated with decreased survival. However, gain of 1q, independent of 1p/16q mutations, seems to be more common and even more tightly associated with poor outcomes, which will likely be a key factor involved with adjuvant therapy considerations. Summary New treatment protocols for Wilms tumor are forthcoming and will include more molecular markers for risk stratification. Other COG studies on more rare renal tumors are underway to further improve survival for patients with these malignancies.

Robotic-assisted simple prostatectomy (RASP) has emerged as a safe and effective treatment option for symptomatic patients with lower urinary tract symptoms related to significant benign prostatic enlargement (BPE) above 80 g. The recent release of the da Vinci SP robotic system (Intuitive, Sunnyvale, CA, USA) continues to advance the minimally invasive nature of robotic surgical technology. We now report our institution’s initial experience performing RASP using the da Vinci SP robotic system. An IRB-approved, retrospective chart review was performed of all patients undergoing robotic-assisted simple prostatectomy using the da Vinci SP surgical system in the treatment of benign prostatic enlargement by a single surgeon from March to June 2019. Pre-operative, intraoperative, and post-operative data were collected for descriptive analysis. A total of 10 men, mean age of 69 ± 4 years, with mean prostate volume of 104 ± 11 g underwent surgery. The robotic cannula and a single assistant port were utilized in all cases. No cases required conversion to a multi-port robotic platform or open approach, nor required the placement of additional assistant ports. No intraoperative or immediate post-operative complications were noted. Mean estimated blood loss was 141 ± 98 mL and operative time was 172 ± 19 min. Mean catheter time was 1.9 ± 1.8 days. One patient reported transient de novo stress urinary incontinence. Single-port RASP is a safe and effective intervention for BPE. The smaller surgical footprint from the device appears to make earlier catheter removal possible. Comparative evaluation with multi-port RASP and other modalities is warranted.

Purpose To evaluate the impact of dedicated reader education on accuracy/confidence of peripheral zone index cancer and anterior prostate cancer (PCa) diagnosis with mpMRI; secondary aim was to assess the ability of readers to differentiate low-grade cancer (Gleason 6 or below) from high-grade cancer (Gleason 7+). Materials and methods Five blinded radiology fellows evaluated 31 total prostate mpMRIs in this IRB-approved, HIPAA-compliant, retrospective study for index lesion detection, confidence in lesion diagnosis (1–5 scale), and Gleason grade (Gleason 6 or lower vs. Gleason 7+). Following a dedicated education program, readers reinterpreted cases after a memory extinction period, blinded to initial reads. Reference standard was established combining whole mount histopathology with mpMRI findings by a board-certified radiologist with 5 years of prostate mpMRI experience. Results Index cancer detection: pre-education accuracy 74.2%; post-education accuracy 87.7% ( p  = 0.003). Confidence in index lesion diagnosis: pre-education 4.22 ± 1.04; post-education 3.75 ± 1.41 ( p  = 0.0004). Anterior PCa detection: pre-education accuracy 54.3%; post-education accuracy 94.3% ( p  = 0.001). Confidence in anterior PCa diagnosis: pre-education 3.22 ± 1.54; post-education 4.29 ± 0.83 ( p  = 0.0003). Gleason score accuracy: pre-education 54.8%; post-education 73.5% ( p  = 0.0005). Conclusions A dedicated reader education program on PCa detection with mpMRI was associated with a statistically significant increase in diagnostic accuracy of index cancer and anterior cancer detection as well as Gleason grade identification as compared to pre-education values. This was also associated with a significant increase in reader diagnostic confidence. This suggests that substantial interobserver variability in mpMRI interpretation can potentially be reduced with a focus on education and that this can occur over a fellowship training year.

Abstract Disclosure: C. Nguyen: None. R. Yong: None. X. Xu: None. Y. Lin: None. S.K. Gunn: None. V. Sutton: None. N. Passoni: None. M. Axelrad: None. J. Bercaw: None. P. Georgiadis: None. P. Austin: None. M. Scheurer: None. D. Nguyen: None. L.P. Karaviti: None. Background: Congenital Adrenal Hyperplasia (CAH) is a condition characterized by a deficiency in the adrenal enzyme, 21-hydroxylase, due to mutations in the CYP21A2 gene. Therapeutic management of CAH attempts to replace deficient hormones and reduce excessive androgen levels. In all cases of classical CAH, surgical intervention is required to restore external genitalia and the urogenital sinus. The timing of the surgical intervention is critical and still remains a challenge to restore the balance and quality of life for these patients. Currently, there is no consensus or data-driven algorithms to help determine the optimal timing for surgery for those who present with different types of classical CAH. Objective: The purpose of the study is to determine whether patients who undergo early or late surgery have better clinical and quality of life outcomes and what factors underlie these differences. Method: A retrospective chart review was conducted of girls under 18 years of age diagnosed with CAH or CAH with salt loss who underwent feminizing genitoplasty surgery at Texas Children’s Hospital (TCH). We collected data including patient demographics, the patient’s age at the time of the initial consult and at the time of surgery, the class of CAH, the grade of virilization; the timing of surgery (early or delayed), the type of surgical treatment, the outcomes, complications, follow-up visits, as well as other factors that influenced the decision-making process regarding the timing of surgery. Results: Analysis of the charts identified 40 girls diagnosed with CAH at TCH between April 2012 and June 2022. The median age at diagnosis was 0.4 months. The three girls were diagnosed with the simple virilizing form of CAH and did not undergo surgery. Thirty-seven girls were diagnosed with the salt-wasting form of CAH, and 29 underwent surgery (78.3%). Of those 29 patients, 15 girls (51.5%) had surgery in the first 12 months of life, 9 (31%) had surgery between 13-24 months, 2 (6.9%) had surgery between 37-48 months, and 3 (10.3%) had surgery > 120 months. Data for Prader staging showed equal proportions of girls who underwent surgery were classified as either Prader stage III (44.8%) or IV (44.8%). No significant differences were found in clinical parameters, complications or compliance. Conclusions: This study provided valuable insights into the management of CAH patients by examining the relationship between the timing of surgery and outcomes. The results can be used to inform data-driven guidelines that will reduce the variability in the current standard of care and improve surgical outcomes for CAH patients. Presentation: 6/1/2024

Erectile dysfunction (ED) is one of the complications associated with pelvic surgery. The significance of ED as a complication following pelvic surgery, especially radical prostatectomy (RP), lies in the negative impact that it has on patients’ sexual and overall life. In the literature, rates of ED following RP range from 25% to 100%. Such variety is associated with pelvic dissection and conservation of neurovascular structures. Another important factor impacting on postoperative ED is the preoperative erectile function of the patient. Advances in the knowledge of pelvic anatomy and pathological mechanisms led to a refinement of pelvic surgical techniques, with attention to the main structures that if damaged compromise erectile function. These improvements resulted in lower postoperative ED rates and better erectile recovery, especially in patients undergoing RP. Furthermore, surgery alone is not sufficient to prevent this complication, and thus, several medical strategies have been tested with the aim of maximizing erectile function recovery. Indeed it seems that prevention of postoperative ED must be addressed by a multimodal approach. The aim of this review is to give a picture of recent knowledge, novel techniques and therapeutic approaches in order to reach the best combination of treatments to reduce the rate of ED after pelvic surgery.

Background: We sought to determine whether formalin disinfection of prostate biopsy needles between cores reduces post-biopsy urinary tract infections (UTIs). Methods: We reviewed a single-surgeon experience of transrectal prostate biopsies from 2010 to 2014. Biopsies were performed in either an operative suite, where 10% formalin was used to disinfect the needle tip between each biopsy core, or an outpatient clinic, where formalin was not used. Our primary outcome was post-biopsy UTI rates, defined as a positive urine culture within 30 days of biopsy. Infection severity was characterized by the need for admission. Patient demographics, prostate size, prior biopsies, prior UTIs, pre-biopsy antibiotics and cultures and post-biopsy cultures were analyzed. Logistic regression was used to assess predictors of post-biopsy UTIs. Statistical significance was defined as P <0.05. Results: A total of 756 patients were included for analysis, including 253 who received formalin disinfection and 503 who did not. Of these, 32 patients (4.2%) experienced post-biopsy UTIs, with 8 requiring admission (all without formalin use). Infection rates were more than double in the group that did not receive formalin (5.2% vs 2.3%, P =0.085). More patients in the formalin group had undergone prior biopsies (73.9% vs 31.8%, P <0.001). On multivariable analysis, prior UTI (odds ratio (OR) 3.77, P =0.006) was a significant predictor for post-biopsy infection, whereas formalin disinfection trended towards a protective effect (OR 0.41, P =0.055). Conclusion: Infectious complications following prostate biopsy may be mitigated by the use of formalin disinfection of the biopsy needle between cores.

Purpose An increasing body of evidence suggests gender differences in the presentation and prognosis of bladder cancer. We aimed to assess the impact of gender on outcomes in patients with primary T1 high-grade (HG) urothelial carcinoma of the bladder (UCB). Methods We retrospectively analysed the data from 916 patients with primary T1HG UCB from 7 tertiary care centres. Patients were treated with transurethral resection of the bladder with or without intravesical instillation therapy (IVT). Univariable and multivariable Cox regression analyses assessed the effect of gender on outcomes. Results Within a median follow-up of 42.8 months, 365 (39.8 %) patients experienced disease recurrence, 104 (11.4 %) progression, 59 (6.4 %) cancer-specific mortality and 190 (20.7 %) mortality of any cause. Overall, 634 (69.2 %) patients received IVT of which 234 (25.5 %) received BCG therapy. Female gender ( n  = 190, 20.7 %) was associated with higher risk of disease recurrence (HR:1.359;1.071–1.724, p  = 0.012) in all patients and in a subgroup of patients treated with BCG therapy (HR:1.717;1.101–2.677, p  = 0.017). There was no difference between genders with regard to disease progression, cancer-specific mortality and any-cause mortality. In multivariable analyses that adjusted for the effects of concomitant carcinoma in situ (CIS), tumour size, number of tumours, and IVT, gender remained an independent predictor for disease recurrence ( p  = 0.026) when analysed in all patients, but not in the subgroup of BCG treated patients ( p  = 0.093). Conclusions In patients with T1HG UCB, female gender is associated with higher risk of disease recurrence, but not with disease progression. This gender disparity may be due to differences in care and/or biology of UCB.