Characteristics and Outcomes of Patients With Spina Bifida in Texas by Patient Age.

INTRODUCTION: Spina bifida (SB) occurs in 3.5/10,000 live births and is associated with significant long-term neurologic and urologic morbidity. We explored the characteristics and outcomes of pediatric patients with SB and the facilities that treat them in Texas. METHODS: We retrospectively reviewed a statewide hospital inpatient discharge database (2013-2021) to identify patients aged <18 y with SB using International Classification of Diseases 9/10 codes. Patients transferred to outside hospitals were excluded to avoid double-counting. Descriptive statistics and chi-square test were performed. RESULTS: Seven thousand five hundred thirty one inpatient hospitalizations with SB were analyzed. Most SB care is provided by a few facilities. Two facilities (1%) averaged >100 SB admissions per year (33% of patients), while 15 facilities (8%) treat 10-100 patients per year (51% of patients). Most facilities (145/193, 75%) average less than one patient per year. Infants tended to be sicker (17% extreme illness severity, P < 0.001). Overall mortality is low (1%), primarily occurring in the neonatal period (8%, P < 0.001). Most admissions are associated with surgical intervention, with 63% of encounters having operating room charges with an average cost of $25,786 ± 24,884. Admissions for spinal procedures were more common among infants, whereas admissions for genitourinary procedures were more common among older patients (P < 0.001). The average length of stay was 8 ± 16 d with infants having the longest length of stay (19 ± 33, P < 0.001). CONCLUSIONS: Patients have significant long-term health needs with evolving pediatric surgical indications as they grow. Pediatric SB care is primarily provided by a small number of facilities in Texas. Longitudinal care coordination of their multidisciplinary surgical care is needed to optimize patient care.

Phenotypic severity scoring system and categorisation for prune belly syndrome: application to a pilot cohort of 50 living patients.

OBJECTIVE: To design a novel system of scoring prune belly syndrome (PBS) phenotypic severity at any presenting age and apply it to a large pilot cohort. PATIENTS AND METHODS: From 2000 to 2017, patients with PBS were recruited to our prospective PBS study and medical records were cross-sectionally analysed, generating individualised RUBACE scores. We designed the pragmatic RUBACE-scoring system based on six sub-scores (R: renal, U: ureter, B: bladder/outlet, A: abdominal wall, C: cryptorchidism, E: extra-genitourinary, generating the acronym RUBACE), yielding a potential summed score of 0-31. The 'E' score was used to segregate syndromic PBS and PBS-plus variants. The cohort was scored per classic Woodard criteria and RUBACE scores compared to Woodard category. RESULTS: In all, 48 males and two females had a mean (range) RUBACE score of 13.8 (8-25) at a mean age of 7.3 years. Segregated by phenotypic categories, there were 39 isolated PBS (76%), six syndromic PBS (12%) and five PBS-plus (10%) cases. The mean RUBACE scores for Woodard categories 1, 2, and 3 were 20.5 (eight patients), 13.8 (25), and 10.6 (17), respectively (P < 0.001). CONCLUSIONS: RUBACE is a practical, organ/system level, phenotyping tool designed to grade PBS severity and categorise patients into isolated PBS, syndromic PBS, and PBS-plus groups. This standardised system will facilitate genotype-phenotype correlations and future prospective multicentre studies assessing medical and surgical treatment outcomes.

Prospective Inclusion of Apparent Diffusion Coefficients in Multiparametric Prostate MRI Structured Reports: Discrimination of Clinically Insignificant and Significant Cancers.

OBJECTIVE: The purpose of this study was to assess the diagnostic performance of prospectively assigned mean apparent diffusion coefficient (ADC) values in multiparametric MRI (mpMRI) structured reports for discriminating clinically insignificant (International Society of Urological Pathology [ISUP] group 1) from clinically significant (ISUP groups 2-5) prostate cancer. MATERIALS AND METHODS: This single-center study included men with abnormal 3-T mpMRI findings (Prostate Imaging Reporting and Data System version 2 score, ≥ 3) who subsequently underwent radical prostatectomy or targeted biopsy with positive results. One of nine radiologists prospectively reported the mean ADC for each lesion during clinical interpretation. Lesions with ADC ≤ 0.700 mm2/s × 10-3 were flagged as concerning for clinically significant prostate cancer. The index lesion at MRI correlated with the site-concordant lesion at targeted biopsy or whole-mount histopathologic analysis. Logistic regression was used to evaluate the utility of mean ADC values for discriminating ISUP grade group 1 from groups 2-5. Diagnostic performance was assessed by ROC AUC. RESULTS: Among the 218 eligible men, those with ISUP group 2-5 lesions had lower mean ADC values than those with ISUP 1 lesions; overall, 0.598 vs 0.803 mm/s2 × 10-3 (p < 0.0001); peripheral zone (PZ), 0.597 vs 0.855 mm/s2 × 10-3 (p < 0.0001); transition zone (TZ), 0.600 vs 0.660 mm/s2 × 10-3 (p = 0.035). The AUC for the PZ was 0.91 and for the TZ was 0.70. The optimal ADC cutoff values were 0.682 mm/s2 × 10-3 for PZ lesions and 0.638 mm2/s × 10-3 for TZ lesions, resulting in sensitivity and specificity of 78% and 94% for the PZ and 72% and 67% for the TZ. CONCLUSION: ADC values estimated prospectively in a clinical setting can help differentiate clinically insignificant from clinically significant prostate cancer, facilitating prebiopsy and pretreatment risk stratification.

Diagnostic Utility of a Likert Scale Versus Qualitative Descriptors and Length of Capsular Contact for Determining Extraprostatic Tumor Extension at Multiparametric Prostate MRI.

OBJECTIVE: The purpose of this study is to determine the reproducibility and diagnostic performance of a Likert scale in comparison with the European Society of Urogenital Radiology (ESUR) criteria and tumor-pseudocapsule contact length (TCL) for the detection of extraprostatic extension (EPE) at multiparametric MRI. MATERIALS AND METHODS: This was a retrospective review of all men who underwent multiparametric MRI followed by prostatectomy between November 2015 and July 2016. Multiparametric 3-T MRI studies with an endorectal coil were independently reviewed by five readers who assigned the likelihood of EPE using a 1-5 Likert score, ESUR criteria, and TCL (> 10 mm). EPE outcome (absent or present) for the index lesion at whole-mount histopathologic analysis was the standard of reference. Odds ratios (ORs) and areas under the ROC curve (Az) were used for diagnostic accuracy. The interreader agreement was determined using a weighted kappa coefficient. A p < 0.05 was considered significant. RESULTS: Eighty men met the eligibility criteria. At univariate analysis, the Likert score showed the strongest association (OR, 1.8) with EPE, followed by prostate-specific antigen level (OR, 1.7), ESUR score (OR, 1.6), and index lesion size (OR, 1.2). At multivariable analysis, higher Likert score (OR, 1.8) and prostate-specific antigen level (OR, 1.6-1.7) were independent predictors of EPE. The Az value for Likert scores was statistically significantly higher (0.79) than that for TCL (0.74; p < 0.01), but not statistically significantly higher than the value for ESUR scores (0.77; p = 0.17). Interreader agreement with Likert (κ = 0.52) and ESUR scores (κ = 0.55) was moderate and slightly superior to that for TCL (κ = 0.43). Except for TCL among inexperienced readers (κ = 0.34), reader experience did not affect interreader agreement. CONCLUSION: A Likert score conveying the degree of suspicion at multiparametric MRI is a stronger predictor of EPE than is either ESUR score or TCL and may facilitate informed decision making, patient counseling, and treatment planning.

Anti-inflammatory use may not negatively impact oncologic outcomes following intravesical BCG for high-grade non-muscle-invasive bladder cancer.

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.

Impact of stage migration and practice changes on high-risk prostate cancer: results from patients treated with radical prostatectomy over the last two decades.

OBJECTIVE: To evaluate the impact of year of surgery on clinical, pathological and oncological outcomes of patients with high-risk prostate cancer. PATIENTS AND METHODS: We evaluated 1 033 patients with clinically high-risk prostate cancer, defined as the presence of at least one of the following risk factors: preoperative prostate-specific antigen (PSA) level >20 ng/mL, and/or clinical stage ≥T3, and/or biopsy Gleason score ≥8. Patients were treated between 1990 and 2013 at a single institution. The year-by-year trends in clinical and pathological characteristics were examined. Multivariable Cox regression analysis was used to test the relationship between year of surgery and oncological outcomes. RESULTS: We observed a decrease over time in the proportion of patients with high-risk disease (preoperative PSA >20 ng/mL or clinical stage cT3). A trend in the opposite direction was seen for biopsy Gleason score ≥8 tumours. We observed a considerable increase in the median number of lymph nodes removed, which was associated with an increased rate of lymph node invasion (LNI). On multivariable Cox regression analysis, year of surgery was associated with a reduced risk of biochemical recurrence (hazard ratio [HR] per 5-year interval 0.90, 95% confidence interval [CI] 0.84-0.96; P = 0.01) and distant metastasis (HR per 5-year interval 0.91, 95% CI 0.83-0.99; P = 0.039), after adjusting for age, preoperative PSA, pathological stage, LNI, surgical margin status, and pathological Gleason score. CONCLUSIONS: In this single-centre study, an increased diagnosis of localized and less extensive high-grade prostate cancer was observed over the last two decades. Patients with high-risk disease who were selected for radical prostatectomy showed better cancer control over time. Better definitions of what constitutes high-risk prostate cancer among contemporary patients are needed.

Detection of prostate cancer with multiparametric MRI (mpMRI): effect of dedicated reader education on accuracy and confidence of index and anterior cancer diagnosis.

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.

Prospective evaluation of a preoperative biomarker panel for prediction of upstaging at radical cystectomy.

OBJECTIVES: To prospectively test whether a panel of biomarkers could identify patients with organ-confined disease likely to be upstaged at radical cystectomy (RC), as retrospective studies have found that cell-cycle- and proliferation-related biomarkers can help improve prognostic accuracy after RC. PATIENTS AND METHODS: We prospectively performed p53, p21, p27, Ki67, and cyclin E1 immunohistochemical staining on transurethral resection of the bladder (TURB) specimens from 87 patients treated with RC for organ-confined urothelial carcinoma of the bladder (UCB). The number of altered biomarkers was categorised as 'favourable' (≤2 altered markers) or 'unfavourable' (>2). RESULTS: Expression of p53, p21, p27, cyclin E1, and Ki67 were altered in 61 (70%), 19 (22%), 26 (30%), four (5%), and 70 (80%) patients, respectively. The median number of positive markers was two. In all, 47 (54%) patients were upstaged when T-stage was considered alone and 49 (56%) when T- and/or N-stage were considered both as upstaging. In multivariable analyses that adjusted for the effects of age, clinical stage, concomitant carcinoma in situ, and time from TURB to RC, an 'unfavourable' biomarker score was independently associated with T-stage upstaging (hazard ratio [HR] 3.3, P = 0.024) but not T- and/or N-stage upstaging (HR 2.76, P = 0.06). Addition of p27, number of positive markers, and biomarker score each increased the discrimination of a base model for prediction of T-stage upstaging (5%, 6%, and 5%, respectively) and T- and/or N-stage upstaging (4%, 6%, and 3%, respectively). CONCLUSIONS: Cell-cycle- and proliferation-related markers in the TURB specimen improve the prediction of upstaging at RC. Such a marker panel may help identify patients with non-muscle-invasive UCB who are clinically under-staged needing RC and patients with muscle-invasive UCB who are likely to be non-organ-confined thereby potentially benefiting from neoadjuvant chemotherapy.

Prognosis of patients with pelvic lymph node (LN) metastasis after radical prostatectomy: value of extranodal extension and size of the largest LN metastasis.

OBJECTIVE: To assess the prognostic role of extranodal extension (ENE) and the size of the largest lymph node (LN) metastasis in predicting early biochemical relapse (eBCR) in patients with LN metastasis after radical prostatectomy (RP). PATIENTS AND METHODS: We evaluated BCR-free survival in men with LN metastases after RP and pelvic LN dissection performed in six high-volume centres. Multivariable Cox regression tested the role of ENE and diameter of largest LN metastasis in predicting eBCR after adjusting for clinicopathological variables. We compared the discrimination of multivariable models including ENE, the size of largest LN metastasis and the number of positive LNs. RESULTS: Overall, 484 patients were included. The median (interquartile range, IQR) follow-up was 16.1 (6-27.5) months. The median (IQR) number of removed LNs was 10 (4-14), and the median (IQR) number of positive LNs was 1 (1-2). ENE was present in 280 (58%) patients, and 211 (44%) had their largest metastasis >10 mm. Patients with ENE and/or largest metastasis of >10 mm had significantly worse eBCR-free survival (all P < 0.01). On multivariable analysis, number of positive LNs (≤2 vs >2) and the diameter of LN metastasis (≤10 vs >10 mm), but not ENE, were significant predictors of eBCR (all P < 0.003). ENE and diameter of LN metastasis increased the area under the curve of a baseline multivariable model (0.663) by 0.016 points. CONCLUSIONS: The diameter of the largest LN metastasis and the number of positive LNs are independent predictors of eBCR. Considered together, ENE and the diameter of the largest LN metastasis have less discrimination than the number of positive LNs.

How to select the right patients for focal therapy of prostate cancer?

PURPOSE OF REVIEW: Just as lumpectomy for breast cancer aims at achieving oncological control with maximal tissue preservation, the concept of focal therapy for prostate cancer has evolved with the aim of controlling disease while preserving urinary and erectile function. Our review aims at describing the evolution of focal therapy in time and the resulting advances in patient selection. RECENT FINDINGS: Originally, focal therapy was developed as an alternative to active surveillance for men with low-risk disease and a minimal burden of cancer. However, with improvement of the diagnostic technologies, the entry criteria for this therapeutic approach are broadening. Since its introduction, focal therapy has evolved from ablation of half or three-quarters of the prostate in men with low-risk disease to only selected cancer foci even if they present with Gleason pattern 4. This development has become possible because of a better understanding of the biology of the index lesion(s) and the improvement of biopsy techniques. Candidates were selected at first with conventional transrectal random prostate biopsies. To overcome the sampling bias of standard techniques, a more thorough multicore sampling utilizing transperineal three-dimensional template mapping biopsies was developed. Today, advances in multiparametric MRI allow for detection and targeted biopsies of high-grade and high-volume lesions. SUMMARY: In light of the less stringent selection criteria, enrollment for focal therapy protocols should encourage patient education on the need of possible subsequent cycles of ablation as well as the need for surveillance of the untreated prostatic tissue, as prostate cancer is transformed into a chronic, manageable condition.

LUMBAR Syndrome and Bladder Exstrophy: A Case Report.

LUMBAR syndrome is rare with a multitude of features that requires a high index of suspicion for timely diagnosis and appropriate management. We present on a newborn female whose untreated segmental infantile hemangioma lead to poor healing of her bladder exstrophy closure. The objective of this report is to describe bladder exstrophy as a urogenital anomaly in patients with LUMBAR syndrome and the importance of balancing management of infantile hemangioma and time to surgery.

PIEZO1 loss-of-function compound heterozygous mutations in the rare congenital human disorder Prune Belly Syndrome.

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.

Obesity is associated with worse outcomes in patients with T1 high grade urothelial carcinoma of the bladder.

PURPOSE: To our knowledge the impact of body mass index on oncologic outcomes in nonmuscle invasive bladder cancer has not been evaluated. We hypothesized that higher body mass index is associated with worse outcomes in patients with clinical primary T1 high grade urothelial carcinoma of the bladder. MATERIALS AND METHODS: We retrospectively analyzed data from 892 patients with primary nonmuscle invasive bladder cancer from 7 centers. Patients were treated with transurethral resection of the bladder with or without intravesical therapy. Body mass index was analyzed as a continuous and a categorical variable (nonobese-body mass index less than 30 kg/m(2) vs obese-body mass index 30 kg/m(2) or greater). Disease progression was defined as the development of T2 or higher tumor stage. RESULTS: Median followup was 42.8 months (IQR 56). Of the patients 44.3% were obese and median body mass index was 29.2 kg/m(2) (IQR 8). On univariable analyses higher body mass index and age were associated with an increased risk of disease recurrence, progression, cancer specific mortality and any cause mortality (all p ≤ 0.001). On multivariable analyses that adjusted for the effects of gender, concomitant carcinoma in situ, tumor size, number of tumors and intravesical therapy, higher body mass index and age remained independent predictors of disease recurrence, progression, cancer specific mortality and any cause mortality (all p <0.05). This study was limited by its design (ie lack of data on repeat transurethral resection of the bladder and intravesical therapy protocol). CONCLUSIONS: Patients diagnosed with clinical T1 high grade urothelial carcinoma of the bladder who are obese have worse cancer specific outcomes compared to their nonobese counterparts. Further work is needed to improve our understanding of clinical T1 high grade outcomes in the growing population of obese patients.

Pathological features of lymph node metastasis for predicting biochemical recurrence after radical prostatectomy for prostate cancer.

PURPOSE: Subclassification of nodal stage may have prognostic value in men with lymph node metastasis at radical prostatectomy. We explored the role of extranodal extension, size of the largest metastatic lymph node and the largest metastasis, and lymph node density as predictors of biochemical recurrence. MATERIALS AND METHODS: We reviewed pathological material from 261 patients with node positive prostate cancer. We examined the predictive value when adding the additional pathology findings to a base model including extraprostatic extension, seminal vesicle invasion, radical prostatectomy Gleason score, prostate specific antigen and number of positive lymph nodes using the Cox proportional hazards regression and Harrell concordance index. RESULTS: The median number of lymph nodes removed was 14 (IQR 9, 20) and the median number of positive lymph nodes was 1 (IQR 1, 2). At a median followup of 4.6 years (IQR 3.2, 6.0) 155 of 261 patients experienced biochemical recurrence. The mean 5-year biochemical recurrence-free survival rate was 39% (95% CI 33-46). Median diameter of the largest metastatic lymph node was 9 mm (IQR 5, 16). On Cox regression radical prostatectomy specimen Gleason score (greater than 7 vs 7 or less), number of positive lymph nodes (3 or greater vs 1 or 2), seminal vesicle invasion and prostate specific antigen were associated with significantly increased risks of biochemical recurrence. On subset analysis metastasis size significantly improved model discrimination (base model Harrell concordance index 0.700 vs 0.655, p = 0.032). CONCLUSIONS: Our study confirms that the number of positive lymph nodes is a predictor of biochemical recurrence in men with node positive disease. The improvement in prognostic value of measuring the metastatic focus warrants further investigation.

Nerve-sparing approach during radical prostatectomy is strongly associated with the rate of postoperative urinary continence recovery.

UNLABELLED: WHAT'S KNOWN ON THE SUBJECT? AND WHAT DOES THE STUDY ADD?: Urinary incontinence and erectile dysfunction are the most bothersome sequelae affecting health-related quality of life in patients treated with radical prostatectomy for prostate cancer. While it has been widely reported that a nerve-sparing approach significantly improves postoperative erectile function, the impact of neurovascular bundle preservation on urinary continence recovery is still a matter of controversy. Our study clearly demonstrates that patients treated with nerve-sparing radical prostatectomy have higher chances of recovering full continence after surgery. The results indicate that, when technically and oncologically feasible, an attempt at a nerve-sparing approach should be planned in order to increase the probability of achieving full continence after radical prostatectomy. OBJECTIVE: To demonstrate that nerve-sparing radical prostatectomy (NSRP) is associated with higher rates of urinary continence (UC) recovery compared with non-nerve-sparing procedures in patients with surgically treated organ-confined prostate cancer. PATIENTS AND METHODS: The study included 1249 patients treated with radical prostatectomy between 2003 and 2010. Patients were divided into three preoperative risk groups: low (PSA < 10 ng/mL, cT1, biopsy Gleason sum ≤ 6), high (cT3 or biopsy Gleason 8-10 or PSA > 20 ng/mL) and intermediate (all the remaining). Postoperative UC recovery was defined as the absence of any protection device. The association between nerve-sparing status and UC recovery was assessed in univariable and multivariable Cox regression analyses after accounting for age at surgery, Charlson Comorbidity Index and preoperative risk group. RESULTS: At a mean follow-up of 42.2 months (range 1-78), 993 patients (79.5%) recovered UC. Overall, UC recovery rate at 1 and 2 years was 76% and 79%, respectively. On univariable Cox regression analysis, age at surgery, preoperative risk group, medical comorbidities and nerve-sparing status were significantly associated with UC recovery (all P ≤ 0.001). On multivariable analysis, age, risk group and nerve-sparing status were also independently associated with UC recovery (all P < 0.003). Patients treated with bilateral NSRP had a 1.8-fold higher chance of full UC recovery. CONCLUSIONS: Patients treated with bilateral NSRP have significantly higher chances of recovering full continence. Therefore, when oncologically and technically feasible, a nerve-sparing procedure should be attempted.

A novel tool to assess the risk of urinary incontinence after nerve-sparing radical prostatectomy.

UNLABELLED: WHAT'S KNOWN ON THE SUBJECT? AND WHAT DOES THE STUDY ADD?: Urinary incontinence is one of the most important morbidities after radical prostatectomy that has detrimental effect on the postoperative quality of life. The present study provides an accurate and dynamic multivariable risk stratification tool that predicts the postoperative urinary incontinence risk after radical prostatectomy based on patient-related as well as surgeon-related variables. OBJECTIVE: To develop a multivariable risk classification tool to estimate postoperative urinary incontinence (UI) risk as UI represents one of the most disabling surgical sequelae after radical prostatectomy (RP). PATIENTS AND METHODS: We evaluated 1311 patients treated with nerve-sparing RP between 2006 and 2010 at our institution. Regression tree analysis was used to stratify patients according to their postoperative UI risk. Kaplan-Meier curve estimates were used to assess the UI rate in the novel UI-risk groups. The discrimination of the novel tool was measured with the area under the curve method. RESULTS: At 3, 6 and 12 months, the UI rates were 44%, 26% and 12%, respectively. Regression tree analysis stratified patients into high risk (International Index of Erectile Function - Erectile Function domain [IIEF-EF] = 1-10), intermediate risk (IIEF-EF > 10 and age ≥ 65 years), low risk (IIEF-EF > 10, age < 65 years and body mass index [BMI] ≥ 25 kg/m(2) ) and very low risk (IIEF-EF > 10, age < 65 years and BMI < 25 kg/m(2) ) groups. The 3-month UI rates in these groups were 37%, 43%, 45% and 48%, respectively. The 6-month UI rates were 19%, 23%, 29% and 34%, respectively. The 12-month UI rates were 7%, 13%, 14% and 15%, respectively (log-rank P < 0.001). The area under the curve was 71%, 70% and 68% at 3, 6 and 12 months, respectively. CONCLUSIONS: We developed the first risk classification tool that predicts patients at high risk of UI after RP. These consisted mainly of individuals who were impotent before RP, elderly and/or overweight. This tool can be used for patient counselling.

Female gender is associated with higher risk of disease recurrence in patients with primary T1 high-grade urothelial carcinoma of the bladder.

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.

Delayed Surgical Management of an Unusual Classic Bladder Exstrophy Variant.

Anterior abdominal wall defects are rare anomalies that can affect multiple organ systems including gastrointestinal, genitourinary, musculoskeletal, and the neurospinal axis. The highly varied, complex anatomy in this patient population creates a challenging reconstruction scenario that merits careful surgical planning. We present an unusual female variant with an anorectal malformation as well as musculoskeletal and genital abnormalities consistent with classic bladder exstrophy in which the urinary bladder, sphincter, and urethra were largely uninvolved.

Lymphatic spread of nodal metastases in high-risk prostate cancer: The ascending pathway from the pelvis to the retroperitoneum.

BACKGROUND: The aim of this study was to map the nodal metastases distribution in patients with high-risk prostate cancer (PCa) treated with extended pelvic lymph node dissection (ePLND) and retroperitoneal lymph node dissection (rLND) at the time of radical prostatectomy (RP). MATERIALS AND METHODS: This prospective mapping study included 19 patients with high-risk PCa (sharing at least two out of the three following parameters: PSA >20 ng/ml, cT3, biopsy Gleason score ≥8). All patients were treated with RP, ePLND (removal of the obturator, hypogastric, external iliac, presacral, and common iliac lymph nodes) and rLND (removal of para-aortal/para-caval and inter-aorto-caval lymph nodes) by a single surgeon. All patients signed an informed consent highlighting the absence of clinical data supporting the benefit of this surgical approach. RESULTS: Overall, 18 out of 19 patients (94.7%) had pelvic lymph node invasion. The most commonly affected pelvic nodal landing site was obturator (88.8%), followed by external iliac (83.3%), common iliac (77%), hypogastric (44.4%), and presacral (33.3%). Moreover, 14 (77.8%) patients also had involvement of retroperitoneal lymph nodes. Only patients with positive common iliac lymph nodes having at least five positive lower pelvic lymph nodes (n = 14), also had invariably positive retroperitoneal lymph nodes. No patients with negative common iliac lymph nodes had positive retroperitoneal lymph nodes. CONCLUSIONS: PCa lymphatic spread ascends from the pelvis up to the retroperitoneum invariably through common iliac lymph nodes. PCa lymphatic spread can be divided in two main levels: pelvic and common iliac plus retroperitoneal lymph nodes.

National Comprehensive Cancer Network practice guidelines 2011: Need for more accurate recommendations for pelvic lymph node dissection in prostate cancer.

PURPOSE: The 2011 NCCN (National Comprehensive Cancer Network) guidelines for prostate cancer recommend pelvic lymph node dissection at radical prostatectomy in all individuals with a nomogram predicted lymph node invasion probability of 2% or greater. We examined the ability of these guidelines to correctly predict lymph node invasion in patients treated with extended pelvic lymph node dissection. MATERIALS AND METHODS: We examined 3,064 consecutive patients treated with radical prostatectomy and extended pelvic lymph node dissection between 2000 and 2010. We formally validated the NCCN guideline nomogram using discrimination, calibration and decision curve analysis as benchmarks. Moreover the performance characteristics of the 2% nomogram cutoff as well as other cutoff values (range 1% to 10%) were tested. RESULTS: Overall 10.0% of patients had lymph node invasion. The discrimination accuracy of the NCCN guideline nomogram was 79.8%, with a maximum underestimation of the lymph node invasion risk of 41.2%. On decision curve analysis the NCCN nomogram fared better than not performing pelvic lymph node dissection in all patients. However, in the prediction range between 0% and 9% the nomogram did not fare better than performing pelvic lymph node dissection in all patients. The use of the 2% cutoff would allow the avoidance of 49.3% of pelvic lymph node dissections, at the cost of missing 20.3% of patients with lymph node invasion. CONCLUSIONS: The NCCN nomogram tends to significantly underestimate the real lymph node invasion rate. Moreover the use of the currently recommended cutoff of 2% to trigger pelvic lymph node dissection might not be appropriate.