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INTRODUCTION AND OBJECTIVE:Two chemotherapeutic agents used widely in pediatric oncology are vincristine (VCR) and doxorubicin (DOX), which cause neuropathy and myopathy, respectively. The study hy...

Data are lacking on the current perception of oncology care among pediatric urologists. Thus, we developed, pilot tested and administered a survey on this topic to SPU (Society for Pediatric Urology) members.Approval for this proposal was granted by SPU leadership prior to developing or distributing the survey instrument. The survey was developed and pilot tested by the PUOWG (Pediatric Urologic Oncology Working Group). Response data were collected and descriptive statistics were used for analysis. Logistic regression analysis was performed to correlate surgeon reported factors with higher volumes of reported oncology surgery.A total of 426 surveys were distributed via email to SPU members and 212 individual surveys (49.8%) were returned with the background/introduction section completed. Of these surveys 200 (94.3%) were completed by practicing pediatric urologists. Overall, 155 respondents (77.5%) reported performing 5 or fewer oncology related surgeries per year and 74.9% reported that less than 25% of renal tumor surgery at their institution was performed through the pediatric urology service. On multivariate analysis the self-reported factors significantly associated with increased oncology surgical volume (more than 5 cases per year) were greater than 50% attendance at institutional tumor board meetings (OR 4.8, 95% CI 1.4-16.9) and practicing at a hospital with a higher volume of renal tumor surgery (OR 2.6, 95% CI 1.2-5.8).Few surveyed pediatric urologists reported performing a high volume of oncology surgery. Respondents expressed interest in ways to increase pediatric urology involvement in oncology care, including opportunities for increased education. Self-reported factors that correlated with higher volume were regular attendance at the institutional pediatric tumor board and practice at a higher volume institution.

Tuberous sclerosis complex is a genetic disorder characterized by the growth of hamartomas in multiple organs. Up to 80% of patients with tuberous sclerosis complex will have at least 1 angiomyolipoma in their lifetime. We describe the incidence and natural history of angiomyolipoma in a pediatric tuberous sclerosis complex population and analyze tumor growth to determine optimal renal imaging intervals in an effort to improve counseling, treatment and followup.We performed a retrospective chart review of all patients with tuberous sclerosis complex from 2004 to 2014. Patients were included if they had a clinical or genetic diagnosis of tuberous sclerosis complex and had undergone at least 1 renal imaging study.A total of 145 patients were analyzed. Median age was 14 years (range 0 to 28). Overall incidence of angiomyolipoma was 50.3%. Median age at first angiomyolipoma detection was 11 years (range 2 to 26). Median yearly angiomyolipoma growth rate stratified by age at first detection was 0.0 mm for patients 0 to 6 years old, 0.9 mm for those 7 to 11 years old, 2.5 mm for those 12 to 16 years old and 1.8 mm for those 17 years old or older. Median yearly angiomyolipoma growth rate stratified by tumor size at first detection was 0.1 mm for tumors 0.6 to 0.9 cm, 1.8 mm for those 1.0 to 1.9 cm and 4.3 mm for those 2.0 to 2.9 cm. A total of 35 patients (24.1%) received mTOR (mammalian target of rapamycin) inhibitors. Eight patients underwent a total of 13 surgical interventions, of whom 2 had previously been treated with mTOR inhibitors. Median patient age at surgical intervention was 18.0 years and median angiomyolipoma size was 5.0 cm.Angiomyolipoma growth in children with tuberous sclerosis complex can be rapid and unpredictable. We recommend yearly renal ultrasound in all patients with tuberous sclerosis complex, with consideration of magnetic resonance imaging in those at risk for rapid growth and future intervention (ie those older than 11 years and/or those with renal angiomyolipomas larger than 2 cm).

INTRODUCTION Testicular microlithiasis (TM) is a condition characterized by calcium deposits within the testis, usually detected incidentally during ultrasonography of the scrotum. TM has been associated with the presence of, and possibly the development of, testicular malignancy. Our aim was to document international clinical management practices for TM and to analyze what factors and perception of risk influence conservative versus active management and follow-up. METHODS European Society for Paediatric Urology (ESPU) and Society for Pediatric Urology (SPU) members were invited to complete an online case-based survey of clinical management practices of TM. Eight cases had a single variable changed each time (classic versus limited TM, unilateral versus bilateral, prior cryptorchidism versus no cryptorchidism) to ascertain the provider's perception of risk. The respondents completed multiple choice questions on initial management, follow-up plan, length and interval of follow-up. Multivariate logistic regression was performed to determine factors associated with decisions on management and follow-up. RESULTS There were 265 respondents to the survey from 35 countries (Table). Median time in practice was 13 years. Factors that were significantly associated with more aggressive initial management (more than counseling on self-examination) included: not yet in independent practice, low volume TM cases per year, those practicing pediatric and adult urology, classic appearance of TM and cryptorchidism. Factors that were significantly associated with urologist follow-up and active investigation included: European practitioners, low TM case volume per year, those practicing both pediatric urology and pediatric surgery, classic TM appearance and a case history of cryptorchidism. Interval and length of follow-up was wide-ranging, with most respondents favoring annual follow-up. CONCLUSION Management of TM varies and a mix of surgeon and case factors significantly influences management strategies. This baseline understanding of the lack of systematic management suggests the need for the development of consensus guidelines and prospective study.

Nephron sparing surgery is the standard of care for many adults with renal tumors and has been described in some children with Wilms tumor. However, beyond case series the data concerning nephron sparing surgery application and outcomes in patients with Wilms tumor are scarce. We examined nephron sparing surgery outcomes and factors associated with its application in children with Wilms tumor.We retrospectively reviewed the 1998 to 2010 SEER database. We identified patients 18 years old or younger with Wilms tumor. Clinical, demographic and socioeconomic data were abstracted, and statistical analysis was performed using multivariate logistic regression (predicting use of nephron sparing surgery limited to unilateral tumors smaller than 15 cm) and Cox regression (predicting overall survival) models.We identified 876 boys and 956 girls with Wilms tumor (mean ± SD age 3.3 ± 2.9 years). Of these patients 114 (6.2%) underwent nephron sparing surgery (unilateral Wilms tumor in 74 and bilateral in 37). Median followup was 7.1 years. Regarding procedure choice, nephron sparing surgery was associated with unknown lymph node status (Nx vs N0, p0.001) and smaller tumor size (p0.001). Regarding survival, only age (HR 1.09, p = 0.002), race (HR 2.48, p = 0.002), stage (HR 2.99, p0.001) and lymph node status (HR 2.17, p = 0.001) predicted decreased overall survival. Survival was not significantly different between children undergoing nephron sparing surgery and radical nephrectomy (HR 0.79, p = 0.58).In children with Wilms tumor included in the SEER database nephron sparing surgery has been infrequently performed. Nephron sparing surgery application is associated with smaller, bilateral tumors and with omission of lymphadenectomy. However, there are no evident differences in application of nephron sparing surgery based on demographic or socioeconomic factors. Despite lymph node under staging, overall survival is similar between patients undergoing nephron sparing surgery and radical nephrectomy.

Tamsulosin is associated with increased passage of ureteral stones in adults but its effectiveness in children is uncertain. We determined the association between tamsulosin and the spontaneous passage of ureteral stones in children.We performed a multi-institutional retrospective cohort study of patients 18 years or younger who presented between 2007 and 2012 with ureteral stones up to 10 mm and who were treated with tamsulosin or oral analgesics alone. The outcome was spontaneous stone passage, defined as radiographic clearance and/or patient report of passage. Subjects prescribed tamsulosin were matched with subjects prescribed analgesics alone, using nearest neighbor propensity score matching to adjust for treatment selection. Conditional logistic regression models were used to estimate the association between tamsulosin and spontaneous passage of ureteral stones, adjusting for stone size and location.Of 449 children with ureteral stones 334 were eligible for inclusion, and complete data were available for 274 patients from 4 institutions (99 receiving tamsulosin, 175 receiving analgesics alone). Following case matching, there were no differences in age, gender, weight, height, stone size or stone location between the 99 subjects prescribed tamsulosin and the 99 propensity score matched subjects prescribed analgesics alone. In the tamsulosin cohort 55% of ureteral stones passed, compared to 44% in the analgesics alone cohort (p=0.03). In multivariate analysis adjusting for stone size and location tamsulosin was associated with spontaneous passage of ureteral stones (OR 3.31, 95% CI 1.49-7.34).The odds of spontaneous passage of ureteral stones were greater in children prescribed tamsulosin vs analgesics alone.

The COG (Children's Oncology Group) currently recommends surveillance for all children and adolescents with clinical stage I testicular germ cell tumors. However, up to 30% of adults with clinical stage I testicular germ cell tumors harbor occult metastatic disease. In adults with clinical stage I nonseminoma some groups advocate a risk stratified approach. Occult metastases were noted in 50% of patients with features such as lymphovascular invasion or embryonal carcinoma predominance in the orchiectomy. However, to our knowledge there are no data on the impact of high risk features in such pubertal children and postpubertal adolescents.We reviewed an institutional testis cancer database for pubertal children and postpubertal adolescents younger than 21 years. We tested the hypothesis that lymphovascular invasion, or 40% or greater embryonal carcinoma in the orchiectomy specimen, would increase the risk of occult metastases, ie relapse during surveillance or positive nodes on retroperitoneal lymph node dissection.We identified 23 patients with a median age of 18.6 years (range 7.1 to 20.9) at diagnosis. Of these patients 14 (60.9%) were on surveillance, 9 (39.1%) underwent primary retroperitoneal lymph node dissection and none received initial chemotherapy. Seven patients (30.4%) had occult metastatic disease. High risk pathological features were found in the orchiectomy specimen in 12 patients (52.2%), including all 12 (52.2%) with 40% or greater embryonal carcinoma and 3 (13.0%) with lymphovascular invasion. Seven patients (58.3%) with high risk features had occult metastatic disease vs none (0%) without high risk features (log rank p = 0.031).Approximately half of pubertal children and postpubertal adolescents with high risk clinical stage I testicular germ cell tumors harbor occult metastatic disease. These results may be useful when discussing prognosis and treatment with patients and families.

Recent investigations described the use of NGAL, a sensitive biomarker for kidney injury, in the setting of ureteropelvic junction obstruction. We prospectively evaluated urinary NGAL levels in the affected renal pelvis and bladder of children with ureteropelvic junction obstruction undergoing unilateral dismembered pyeloplasty. Our hypothesis was that higher NGAL in the kidney and bladder would correlate with decreased ipsilateral differential function.We performed a prospective cohort study in patients treated with unilateral dismembered pyeloplasty from 2010 to 2012. Urine was obtained intraoperatively from the bladder and obstructed renal pelvis. A control population of unaffected children was recruited to provide a voided bladder specimen. Bladder NGAL levels were compared between the study and control populations. We tested our study hypothesis by correlating bladder and renal pelvic NGAL levels with the differential renal function of the affected kidney.A total of 61 patients with a median age at surgery of 1.62 years (range 0.12 to 18.7) were enrolled in the study. Median bladder NGAL was 18.6 ng/mg (range 1.4-1,650.8) and median renal pelvic NGAL was 26.2 ng/mg (range 1.2-18,034.5, p = 0.004). Median bladder NGAL was significantly higher than in controls (p = 0.004). The correlation of bladder and renal pelvic NGAL with differential renal function was r = -0.359 (p = 0.004) and r = -0.383 (p = 0.002), respectively.Bladder NGAL is increased in children with ureteropelvic junction obstruction. Renal pelvic and bladder normalized urinary NGAL levels correlate inversely with the relative function of the affected kidney in cases of unilateral ureteropelvic junction obstruction.

While the timely diagnosis and management of pediatric torsion can lead to testicular salvage, limited data exist on rates of orchiopexy vs orchiectomy and associated factors. Thus, we examined the Pediatric Health Information System database for torsion outcomes and demographics at American pediatric hospitals.Using the Pediatric Health Information System database we performed a 7-year retrospective cohort study in children 1 to 17 years old with a primary ICD-9 diagnosis of torsion, assessing CPT codes for orchiopexy and orchiectomy. Data were analyzed with SPSS®, version 17.0.Of 2,876 patients who underwent surgery for an ICD-9 diagnosis code of testicular torsion 918 (31.9%) underwent orchiectomy at a mean age of 10.7 years and 1,958 (68.1%) underwent orchiopexy at a mean age of 12.6 years (p0.0001). In the age groups 1 to 9, 10 to 13 and 14 years or greater 274 (49.9%), 311 (29.4%) and 333 patients (26.2%), respectively, underwent orchiectomy. A higher orchiectomy rate was seen at age 1 to 9 vs 10 years or greater. Torsion and orchiectomy rates did not vary by season or geographic region. A higher orchiectomy rate was seen in black vs white children (37.6% vs 28.1%) and in patients without vs with private insurance (36.7% vs 27.0%). Multivariate analysis revealed an association of age (p0.0001), race (p0.0001) and insurance status (p0.001) with orchiectomy.Nationally an average of 32% of the 411 pediatric torsion cases explored annually result in orchiectomy. Identified factors increasing the orchiectomy risk included age 1 to 9 years, black race and lack of private insurance. Efforts should continue to identify modifiable variables that can increase testicular salvage in patients with testicular torsion.

We reviewed preoperative, and initial and final postoperative urodynamic testing in consecutive children undergoing bladder neck sling without augmentation for neurogenic urinary incontinence to determine if progressive loss of compliance occurs.We assessed consecutive patients with neurogenic outlet incompetence who underwent 360-degree tight fascial wrap around the bladder neck with appendicovesicostomy but no augmentation. This population comprised all patients undergoing outlet surgery between 2002 and 2007. Inclusion criteria were initial urodynamic test within 1 year postoperatively and final urodynamic test at least 18 months postoperatively.A total of 26 patients met inclusion criteria. Most patients (73%) had an acontractile bladder with detrusor pressures less than 25 cm H(2)O preoperatively. Initial postoperative urodynamic test at a mean of 7 months was most predictive of subsequent urodynamic findings. Eight patients (31%) had increased detrusor pressures and/or uninhibited contractions postoperatively. Six patients increased anticholinergic therapy dose. At a mean of 39 months urodynamic patterns were either stable or improved in all patients.Progressive compliance loss was not observed after bladder neck sling without augmentation. Postoperative increases in detrusor pressure and/or uninhibited contractions within 1 year postoperatively should prompt review of anticholinergic therapy rather than enterocystoplasty.

The incidence of port site hernia following adult laparoscopy is 0.1% to 3.0%. There are no known published reports concerning hernia incidence or related factors after pediatric urological laparoscopic interventions. We present our experience with port site incisional hernias following pediatric urological laparoscopy.We reviewed all pediatric urological laparoscopic procedures performed at Children's Medical Center Dallas from 2000 to 2008. A total of 261 cases were identified with followup available in 218 (83.5%). In 187 cases there were sufficient data to evaluate outcomes for each port site separately, and compare the size, location and fascial closure status to hernia development.Median patient age was 6.1 years (range 0.4 to 18.8). A total of 218 patients had a median followup of 5.7 months (range 0.2 to 83.4). Seven hernias (3.2%) were diagnosed at a median of 1.2 months (range 0.1 to 15.1) postoperatively. Patients with hernia were younger than those without hernia, at 1.1 years (range 0.5 to 3.9) vs 6.2 years (0.4 to 18.8, p = 0.04). We analyzed 571 port sites in 187 cases. In 385 ports (67.4%) the fascia was closed. Hernia developed in 4 of 385 ports (1.0%) that were closed and in 3 of 186 (1.6%) that were not closed. No significant relationship was observed between hernia development and port size or location.The incidence of port site hernia after pediatric urological laparoscopy was 3.2%, similar to the reported incidence in adults. While development of hernia after pediatric urological laparoscopy is rare, it is more likely to occur in infants. Due to the low incidence of this complication, it is difficult to draw conclusions regarding contributing factors.