Long-term outcomes and patterns of relapse in patients with bilateral Wilms tumor or bilaterally predisposed unilateral Wilms tumor, a report from the COG AREN0534 study.

The objective of this study is to report the long-term timing and patterns of relapse for children enrolled in Children's Oncology Group AREN0534, a multicenter phase III clinical trial conducted from 2009 to 2015. Participants included children with bilateral Wilms tumor (BWT) or unilateral WT with genetic predisposition to develop BWT followed for up to 10 years. Smoothed hazard (risk) functions for event-free survival (EFS) were plotted so that the timing of events could be visualized, both overall and within pre-specified groups. Two hundred and twenty-two children (190 BWT and 32 unilateral WT with BWT predisposition) were followed for a median of 8.6 years. Fifty events were reported, of which 48 were relapse/progression. The overall 8-year EFS was 75% (95% confidence interval: 69%-83%). The highest risk for an EFS event was immediately after diagnosis with a declining rate over 2 years. A second peak of events was observed around 4 years after diagnosis, and a small number of events were reported until the end of the follow-up period. In subset analyses, later increases in risk were more commonly observed in patients with female sex, anaplastic histology, negative lymph nodes or margins, and favorable histology Wilms tumor patients with post-chemotherapy intermediate risk. Among relapses that occurred after 2 years, most were to the kidney. These patterns suggest that late events may be second primary tumors occurring more commonly in females, although more investigation is required. Clinicians may consider observation of patients with BWT beyond 4 years from diagnosis.

Prognostic impact of lymph node involvement and loss of heterozygosity of 1p or 16q in stage III favorable histology Wilms tumor: A report from Children's Oncology Group Studies AREN03B2 and AREN0532.

INTRODUCTION: The prognostic impact of positive lymph nodes (LN+) and/or singular loss of heterozygosity (LOH) of 1p or 16q were assessed in children with stage III favorable histology Wilms tumor (FHWT) enrolled on AREN0532 or AREN03B2 alone. PATIENTS AND METHODS: A total of 635 stage III FHWT vincristine/dactinomycin/doxorubicin (DD4A)-treated patients met inclusion criteria. Event-free survival (EFS) and overall survival are reported overall and by LN sampling, LN status, LOH 1p, LOH 16q, and a combination of LN status and singular LOH. Patients with unknown or positive combined LOH of 1p and 16q status and AREN03B2-only patients with unknown outcomes or treatment other than DD4A were excluded. RESULTS: EFS did not differ by study, supporting pooling. Lack of LN sampling (hazard ratio [HR], 2.12; p = .0037), LN positivity (HR, 2.78; p = .0002), LOH 1p (HR, 2.18; p = .0067), and LOH 16q (HR, 1.72; p = .042) were associated with worse EFS. Compared with patients with both LN- and LOH-, those with negative nodes but positive LOH 1p or 16q and those with LN+ but LOH- for 1p or 16q had significantly worse EFS (HR, 3.05 and 3.57, respectively). Patients positive for both LN and LOH had the worst EFS (HR, 6.33; overall group factor, p < .0001). CONCLUSION: Findings confirm LN+ status as an adverse prognostic factor amplified by presence of singular LOH 1p or 16q, supporting study of intensified therapy for patients with LN+ in combination with singular LOH in a prospective clinical trial.

Current surgical approaches to pediatric renal tumors.

Pediatric renal tumors are among the most common pediatric solid malignancies. Surgical resection is a key component in the multidisciplinary therapy for children with kidney tumors. Therefore, it is imperative that surgeons caring for children with renal tumors fully understand the current standards of care in order to provide appropriate surgical expertise within this multimodal framework. Fortunately, the last 60 years of international, multidisciplinary pediatric cancer cooperative group studies have enabled high rates of cure for these patients. This review will highlight the international surgical approaches to pediatric patients with kidney cancer to help surgeons understand the key differences and similarities between the European (International Society of Pediatric Oncology) and North American (Children's Oncology Group) recommendations.

Bilateral Wilms tumor with anaplasia: A report from the Children's Oncology Group Study AREN0534.

INTRODUCTION: The purpose of this study is to examine the outcomes in children with anaplastic bilateral Wilms tumor (BWT) from study AREN0534 in order to define potential prognostic factors and areas to target in future clinical trials. METHODS: Demographic and clinical data from AREN0534 study patients with anaplasia (focal anaplasia [FA], or diffuse anaplasia [DA]) were compared. Event-free survival (EFS) and overall survival (OS) were reported using Kaplan-Meier estimation with 95% confidence bands, and differences in outcomes between FA and DA compared using log-rank tests. The impact of margin status was analyzed. RESULTS: Twenty-seven children who enrolled on AREN0534 had evidence of anaplasia (17 DA, 10 FA) in at least one kidney and were included in this analysis. Twenty-six (96%) had BWT. Nineteen percent had anaplastic histology in both kidneys (four of 17 DA, and one of 10 FA). Forty-six percent with BWT had bilateral nephron-sparing surgery (NSS); one child who went off protocol therapy, eventually required bilateral completion nephrectomies. Median follow-up for EFS and OS was 8.6 and 8.7 years from enrollment. Four- and 8-year EFS was 53% [95% confidence interval (CI): 34%-83%] for DA; 4-year EFS was 80% [95% CI: 59%-100%], and 8-year EFS 70% [95% CI: 47%-100%] for FA. Three out of 10 children with FA and eight out of 17 children with DA had events. EFS did not differ statistically by margin status (p = .79; HR = 0.88). Among the six children who died (five DA, one FA), all experienced prior relapse or progression within 18 months. CONCLUSION: Events in children with DA/FA in the setting of BWT occurred early. Caution should be taken about interpreting the impact of margin status outcomes in the context of contemporary multimodal therapy. Future targeted investigations in children with BWT and DA/FA are needed.

Treatment and Outcomes of Congenital Ovarian Cysts A Study by the Canadian Consortium for Research in Pediatric Surgery (CanCORPS).

OBJECTIVE: We conducted a multicenter study to assess treatments and outcomes in a national cohort of infants with congenital ovarian cysts. SUMMARY BACKGROUND DATA: Wide variability exists in the treatment of congenital ovarian cysts. The effects of various treatment strategies on outcomes, specifically ovarian preservation, are not known. METHODS: Female infants diagnosed with congenital intra-abdominal cysts between 2013 and 2017 at 10 Canadian pediatric surgical centers were retrospectively evaluated. Sonographic characteristics, median time to cyst resolution, incidence of ovarian preservation, and predictors of surgery were evaluated. Subgroup analyses were performed in patients with complex cysts and cysts ≥40 mm in diameter. RESULTS: The study population included 189 neonates. Median gestational age at diagnosis and median maximal prenatal cyst diameter were 33 weeks and 40 mm, respectively. Cysts resolved spontaneously in 117 patients (62%), 14 (7%) prenatally, and the remainder at a median age of 124 days. Intervention occurred in 61 patients (32%), including prenatal aspiration (2, 3%), ovary sparing resection (14, 23%), or oophorectomy (45, 74%). Surgery occurred at a median age of 7.4weeks. Independent predictors of surgery included postnatal cyst diameter ≥40 mm [odds ratio (OR) 6.19, 95% confidence interval (CI) 1.66-35.9] and sonographic complex cyst character (OR 63.6, 95% CI 10.9-1232). There was no significant difference in the odds of ovarian preservation (OR 3.06, 95% CI 0.86 -13.2) between patients who underwent early surgery (n = 22) and those initially observed for at least 3 months (n = 131). CONCLUSIONS: Most congenital ovarian cysts are asymptomatic and spontaneously resolve. Early surgical intervention does not increase ovarian preservation.

Technical advances in the surgical management of Wilms tumors in children.

Surgery is one of the cornerstones of Wilms tumor treatment. In this article, we present technical advancements that are finding their way into the armamentarium of pediatric cancer surgeons. We discuss the current approaches, challenges, opportunities, and future directions of minimally invasive surgery (laparoscopic and robotics), image-guided surgery, and fluorescence-guided surgery. Furthermore, we discuss the use of intraoperative ultrasonography, as well as the use of new techniques to improve the quality of lymph node sampling.

Survival effect of complete surgical resection of the primary tumor in patients with metastatic, high-risk neuroblastoma in a large Canadian cohort.

PURPOSE: To determine whether extent of surgical resection of the primary tumor correlates with survival in patients with International Neuroblastoma Staging System (INSS) stage 4, high-risk neuroblastoma. METHODS: Data were extracted for patients with newly diagnosed INSS stage 4, high-risk neuroblastoma between 2001 and 2019 from the national Cancer in Young People in Canada (CYPC) database. Complete resection was defined as gross total resection of primary tumor based on operative reports. Primary endpoints were 3 and 5-year event-free (EFS) and overall survival (OS). Survival analyses were completed using log-rank test and Cox proportional hazards regression including covariates of age, sex, decade of treatment (2001-2009 vs. 2010-2019), immunotherapy, and tandem stem cell transplant (SCT). RESULTS: One-hundred and forty patients with complete surgical data were included. On univariate analysis, 3-year EFS and OS for patients that had complete versus incomplete resection was 71% (95% CI 57-80%) vs. 48% (36-60%) and 86% (75-93%) vs. 64% (51-74%), p = .008 and p = .002, respectively. 5-year EFS and OS for patients with complete resection also demonstrated significantly improved survival. On Cox Proportional Hazards models adjusted for age, immunotherapy, tandem SCT, and surgical resection, only complete resection was associated with statistically significant improved 3 year EFS and OS, HR = 0.48 (0.29-0.81; p = .006) and HR = 0.42 (0.24-0.73; p = .002). CONCLUSIONS: In a large Canadian INSS stage 4 high-risk neuroblastoma cohort, complete surgical resection was associated with increased EFS and OS. Within the constraints of a retrospective study, these results suggest that the ability to achieve primary tumor complete resection in patients with metastatic high-risk disease is associated with improved survival.

Development and implementation of a postgraduate medical education-wide initiative in quality improvement and patient safety.

BACKGROUND: Quality improvement and patient safety (QIPS) have been assigned a higher profile in CanMEDS 2015, CanMEDS-Family Medicine 2017 and new accreditation standards, prompting an initiative at Dalhousie University to create a vision for integrating QIPS into postgraduate medical education. OBJECTIVE: The purpose of this study is to describe the implementation of a QIPS strategy across residency education at Dalhousie University. METHODS: A QIPS task force was formed, and a literature review and needs assessment survey were completed. A needs assessment survey was distributed to all Dalhousie residency programme directors. 12 programme directors were interviewed individually to collect additional feedback. The results were used to develop a 'road map' of recommendations with a graduated timeline. RESULTS: A task force report was released in February 2018. 46 recommendations were developed with a timeframe and responsible party identified for each. Implementation of the QIPS strategy is underway, and evaluation and challenges faced will be described. CONCLUSIONS: We have developed a multiyear strategy that is available to provide guidance and support to all programmes in QIPS. The development and implementation of this QIPS framework may serve as a template for other institutions who seek to integrate these competencies into residency training.

Incidence and management of pleural effusions in patients with Wilms tumor: A Pediatric Surgical Oncology Research Collaborative study.

Wilms tumor (WT) is the most common renal malignancy in children. Children with favorable histology WT achieve survival rates of over 90%. Twelve percent of patients present with metastatic disease, most commonly to the lungs. The presence of a pleural effusion at the time of diagnosis of WT may be noted on staging imaging; however, minimal data exist regarding the significance and prognostic importance of this finding. The objectives of our study are to identify the incidence of pleural effusions in patients with WT, and to determine the potential impact on oncologic outcomes. A multi-institutional retrospective review was performed from January 2009 to December 2019, including children with WT and a pleural effusion on diagnostic imaging treated at Pediatric Surgical Oncology Research Collaborative (PSORC) participating institutions. Of 1259 children with a new WT diagnosis, 94 (7.5%) had a pleural effusion. Patients with a pleural effusion were older than those without (median 4.3 vs 3.5 years; P = .004), and advanced stages were more common (local stage III 85.9% vs 51.9%; P < .0001). Only 14 patients underwent a thoracentesis for fluid evaluation; 3 had cytopathologic evidence of malignant cells. Event-free and overall survival of all children with WT and pleural effusions was 86.2% and 91.5%, respectively. The rate and significance of malignant cells present in pleural fluid is unknown due to low incidence of cytopathologic analysis in our cohort; therefore, the presence of an effusion does not appear to necessitate a change in therapy. Excellent survival can be expected with current stage-specific treatment regimens.

Inflammatory myofibroblastic tumor: A multi-institutional study from the Pediatric Surgical Oncology Research Collaborative.

Inflammatory myofibroblastic tumor (IMT) is a mesenchymal neoplasm of intermediate malignancy. We describe the largest cohort of IMT patients to date, aiming to further characterize this rare, poorly understood tumor. This is a multi-institutional review of IMT patients ≤39 years, from 2000 to 2018, at 18 hospitals in the Pediatric Surgical Oncology Research Collaborative. One hundred and eighty-two patients were identified with median age of 11 years. Thirty-three percent of tumors were thoracic in origin. Presenting signs/symptoms included pain (29%), respiratory symptoms (25%) and constitutional symptoms (20%). Median tumor size was 3.9 cm. Anaplastic lymphoma kinase (ALK) overexpression was identified in 53% of patients. Seven percent of patients had distant disease at diagnosis. Ninety-one percent of patients underwent resection: 14% received neoadjuvant treatment and 22% adjuvant treatment. Twelve percent of patients received an ALK inhibitor. Sixty-six percent of surgical patients had complete resection, with 20% positive microscopic margins and 14% gross residual disease. Approximately 40% had en bloc resection of involved organs. Median follow-up time was 36 months. Overall 5-year survival was 95% and 5-year event-free survival was 80%. Predictors of recurrence included respiratory symptoms, tumor size and distant disease. Gross or microscopic margins were not associated with recurrence, suggesting that aggressive attempts at resection may not be warranted.

The transition of young adults with lifelong urological needs from pediatric to adult services: An international children's continence society position statement.

INTRODUCTION: Children with urinary tract disorders managed by teams, or individual pediatricians, urologists, nephrologists, gastroenterologists, neurologists, psychologists, and nurses at some point move from child-centered to adult-centered health systems. The actual physical change is referred to as the transfer whilst the process preceding this move constitutes transition of care. Our aims are twofold: to identify management and health-service problems related to children with congenital or acquired urological conditions who advance into adulthood and the clinical implications this has for long-term health and specialist care; and, to understand the issues facing both pediatric and adult-care clinicians and to develop a systems-approach model that meets the needs of young adults, their families and the clinicians working within adult services. METHODS: Information was gleaned from presentations at an International Children's Continence Society meeting with collaboration from the International Continence Society, that discussed problems of transfer and transitioning such children. Several specialists attending this conference finalized this document identifying issues and highlighting ways to ease this transition and transfer of care for both patients and practitioners. RESULTS: The consensus was, urological patients with congenital or other lifelong care needs, are now entering adulthood in larger numbers than previously, necessitating new planning processes for tailored transfer of management. Adult teams must become familiar with new clinical problems in multiple organ systems and anticipate issues provoked by adolescence and physical growth. During this period of transitional care the clinician or team assists young patients to build attitudes, skills and understanding of processes needed to maximize function of their urinary tract-thus taking responsibility for their own healthcare needs. Preparation must also address, negotiating adult health care systems, psychosocial, educational or vocational issues, and mental wellbeing. CONCLUSIONS: Transitioning and transfer of children with major congenital anomalies to clinicians potentially unfamiliar with their conditions requires improved education both for receiving doctors and children's families. Early initiation of the transition process should allow the transference to take place at appropriate times based on the child's development, and environmental and financial factors. Neurourol. Urodynam. 36:811-819, 2017. © 2016 Wiley Periodicals, Inc.

Does intracytoplasmic sperm injection pose an increased risk of genitourinary congenital malformations in offspring compared to in vitro fertilization? A systematic review and meta-analysis.

PURPOSE: We systematically reviewed and performed a meta-analysis of the genitourinary congenital malformation rate after conception by intracytoplasmic sperm injection compared to in vitro fertilization. MATERIALS AND METHODS: We systematically reviewed studies to compare perinatal outcomes between children conceived by intracytoplasmic sperm injection vs in vitro fertilization. Studies showing genitourinary congenital malformation rates were included. We assessed the risk of bias, focusing on the quality of genitourinary congenital malformation reporting and analysis targeted at singletons. Meta-analysis was done using a random effects model for 3 outcomes, including overall genitourinary congenital malformation, hypospadias and cryptorchidism. Sensitivity analysis was also performed in only studies at low risk for bias. RESULTS: The initial search yielded 1,482 articles. We performed a full text review of 111 of these studies, of which 22 met inclusion criteria for systematic review. Meta-analysis of intracytoplasmic sperm injection and in vitro fertilization in 12,270 and 24,240 cases, respectively, revealed that intracytoplasmic sperm injection was associated with a significantly higher rate of overall genitourinary congenital malformation compared to in vitro fertilization (OR 1.27, 95% CI 1.02-1.59, p = 0.04). However, when including only 4 studies at low risk for bias with a total of 7,727 and 14,308 intracytoplasmic sperm injection and in vitro fertilization cases, respectively, the difference was not significant (OR 1.28, 95% CI 1.00-1.64, p = 0.05). There was no statistically significant difference in the rate of hypospadias (OR 1.21, 95% CI 0.87-1.69) or cryptorchidism (OR 1.39, 95% CI 0.97-2.00) between males conceived by intracytoplasmic sperm injection vs in vitro fertilization. On all analyses there was no significant statistical heterogeneity between studies (I(2) = 0). CONCLUSIONS: Intracytoplasmic sperm injection is associated with a slightly higher risk of genitourinary malformation in offspring than in vitro fertilization. However, when only higher quality studies were analyzed, the difference was not significant. The hypospadias and cryptorchidism rates in offspring are similar for the 2 conception methods.

Wilms Tumor.

Wilms tumor (WT), or nephroblastoma, is the most common primary malignant renal tumor of childhood. It is an embryonal tumor that develops from remnants of immature kidney. There are approximately 500 new WT cases diagnosed in the United States every year. Advances in multimodal therapy including surgery, chemotherapy, and radiation therapy given according to risk stratification have allowed most patients to achieve survival rates in excess of 90%.

Diagnosis, Classification, and Contemporary Management of Undescended Testicles.

This article provides a comprehensive review regarding undescended testicles and other related conditions. We have included background information summarizing variable clinical presentations, epidemiology, and the implications of undescended testis (UDT) on fertility and malignancy risk. This article has an emphasis on the approach to diagnosis and surgical management for the UDT. The purpose of this review is to provide readers with useful clinical tools for assessing and treating patients with cryptorchidism.

Outcomes according to treatment using an established protocol in patients with bilateral Wilms' tumor: A national Canadian population-based study.

BACKGROUND: Bilateral Wilms tumor (BWT) is a rare entity. The goal of this study is to report outcomes (overall and event-free survival, OS/EFS) of BWT in a large cohort representative of the Canadian population since 2000. We focused on the occurrence of late events (relapse or death beyond 18 months), as well as outcomes of patients treated following the only protocol specifically designed for BWT to date, AREN0534, compared to patients treated following other therapeutic schemes. METHODS: Data was obtained for patients diagnosed with BWT between 2001 and 2018 from the Cancer in Young People in Canada (CYP-C) database. Demographics, treatment protocols, and dates for events were collected. Specifically, we examined outcomes of patients treated according to the Children's Oncology Group (COG) protocol AREN0534 since 2009. Survival analysis was performed. RESULTS: 57/816 (7%) of patients with Wilms tumor had BWT during the study period. Median age at diagnosis was 2.74 years (IQR 1.37-4.48) and 35 (64%) were female; 8/57 (15%) had metastatic disease. After a median follow-up of 4.8 years (IQR 2.8-5.7 years, range 0.2-18 years), OS and EFS were 86% (CI 73-93%) and 80% (CI 66-89%), respectively. Less than 5 events were recorded after 18 months from diagnosis. Since 2009, patients treated according to the AREN0534 protocol had a statistically significant higher OS compared to patients treated with other protocols. CONCLUSIONS: In this large Canadian cohort of patients with BWT, OS and EFS compared favorably to the published literature. Late events were rare. Patients treated according to a disease-specific protocol (AREN0534) had improved overall survival. TYPE OF STUDY: Original article. LEVEL OF EVIDENCE: Level IV.

Geospatial analysis of hypospadias and cryptorchidism prevalence rates based on postal code in a Canadian province with stable population.

INTRODUCTION: Hypospadias and cryptorchidism are hormone-mediated malformations that occur during male development. Prevalence rates of hypospadias and cryptorchidism are thought to be increasing worldwide. In-utero exposure to endocrine-disrupting chemicals (EDCs) may have a role in the occurrence of these malformations. Our group has reported significant clustering of hypospadias and cryptorchidism at the county level in areas of intense agricultural activity in the Canadian province of Nova Scotia (NS). Finer scale spatial analysis has shown clustering near urban centres. OBJECTIVES: The objectives of the study were: 1) to perform a granular geospatial analysis of hypospadias and cryptorchidism prevalence, at the postal code level, of all babies born in NS over a 26-year period; and 2) to determine whether there is spatial correlation between these conditions and industries linked to toxic output. STUDY DESIGN: Cases of hypospadias and cryptorchidism were identified based on ICD-10 codes from the Nova Scotia Atlee Perinatal Database with records of all live births in NS between 1988 and 2013. Data were geocoded and mapped based on the three first digits of the maternal postal code (Forward Sortation Area [FSA]). Regional prevalence of congenital anomalies was calculated for each of the 77 FSAs. To identify statistically significant high and low prevalence clusters for each anomaly, Local Morans I was used on the spatial data. Geospatial point data was created for industries linked to toxic output and correlation between clusters of malformations and proximity to these industries was assessed. RESULTS: During the study period, there were 1045 cases of hypospadias and 993 cases of cryptorchidism. Both hypospadias and cryptorchidism demonstrated statistically significant areas of high prevalence clusters. There was no significant spatial correlation between the local clustering of the congenital malformations and proximity to toxic industries. DISCUSSION AND CONCLUSION: Our study shows heterogeneity in the distribution of hypospadias and cryptorchidism, which is consistent with previously published works. In this follow-up, granular geospatial analysis of hypospadias and cryptorchidism prevalence in an area with stable population, we did not confirm the previous findings of high clustering in areas of intense agricultural activity. Furthermore, our analysis did not find high clustering of the congenital malformations in areas near toxic industries to support a clear environmental role in their development. Some of the limitations include underdiagnosis of hypospadias and cryptorchidism (as they both present with a clinical spectrum and are non-life threatening), and limited data currently available on the route of exposure to EDC industries in Nova Scotia.

Early postoperative ultrasound after open pyeloplasty in children with prenatal hydronephrosis helps identify low risk of recurrent obstruction.

PURPOSE: Prediction of successful pyeloplasty can be challenging, particularly in asymptomatic children treated for worsening prenatally diagnosed hydronephrosis. We evaluated early noninvasive objective predictors of success in this population. MATERIALS AND METHODS: We reviewed patients who underwent pyeloplasty for asymptomatic, prenatally detected, worsening hydronephrosis treated between 2000 and 2010 with followup greater than 1 year. For all patients renal pelvis anteroposterior diameter in a mid transverse view and Society for Fetal Urology hydronephrosis grade were evaluated preoperatively and 3 to 4 months postoperatively. Aside from subjective evaluation based on change in hydronephrosis grade, we estimated the percentage of improvement in anteroposterior diameter (preoperative diameter--postoperative diameter/preoperative diameter). Repeat intervention after pyeloplasty arbitrarily defined failure. Patients were categorized into 3 groups, ie no failure on followup ultrasound (group 1), no failure with postoperative nuclear scan to document success (group 2) and failed pyeloplasty (group 3). ROC curves were plotted to correlate 4 variables with failure, namely preoperative anteroposterior diameter, postoperative anteroposterior diameter, percent improvement in anteroposterior diameter and subjective change in grade. RESULTS: Of 229 patients 192 (84%) who met inclusion criteria had ultrasound at 3 to 4 months postoperatively available. Mean age at surgery was 19 months and mean ± SD followup was 49.9 ± 27.7 months. Percent improvement in anteroposterior diameter and postoperative anteroposterior diameter measure were the most reliable variables to predict failure (AUC 0.88 and 0.86, respectively, p <0.0001), whereas preoperative anteroposterior diameter and subjective changes in grade were not good predictors (AUC 0.52, p >0.05). A percent improvement in anteroposterior diameter of 38% or greater or postoperative anteroposterior diameter of 11.5 mm or less was associated with success, with sensitivity of 100% and specificity of 61% and 55%, respectively. CONCLUSIONS: Percent improvement in anteroposterior diameter and postoperative pelvic anteroposterior diameter measure can provide objective guidance as to which patients need closer monitoring during followup, and can help select children at low risk for repeat intervention after pyeloplasty.

Nephron sparing surgery for unilateral Wilms tumor in children with predisposing syndromes: single center experience over 10 years.

PURPOSE: Unilateral Wilms tumors associated with predisposing syndromes are treated with preoperative chemotherapy followed by surgical resection. We describe our experience with nephron sparing surgery for Wilms tumor in this population at risk for metachronous lesions. MATERIALS AND METHODS: We conducted a retrospective review of all children with a predisposing syndrome who underwent nephrectomy for malignancy during a 10-year period (2000 to 2010). Data collected included age, mode of detection, tumor size, treatment, pathology results, followup time and recurrence episodes. RESULTS: From 2000 to 2010, 13 of 75 (19%) patients treated for Wilms tumor were diagnosed with predisposing syndrome(s). Eight patients with unilateral tumors were treated and had a mean age at diagnosis of 27 months (range 7 months to 9 years). Beckwith-Wiedemann syndrome, isolated hemihyperplasia, WAGR (Wilms tumor, Aniridia, Genitourinary abnormalities, mental Retardation) syndrome and isolated 11p13 deletion were the underlying diagnoses in 3, 2, 2 and 1 patient, respectively. All but 2 patients were diagnosed by screening ultrasound and 5 underwent preoperative chemotherapy. Median tumor size at surgery was 2.5 cm (range 1 to 13). Nephron sparing surgery was performed in 6 of 8 patients. Pathological study showed favorable histology Wilms tumor and nephrogenic rests in 6 and 2 patients, respectively. After a mean followup of 36 months (range 6 to 72) no recurrences were documented and all children had normal creatinine levels. CONCLUSIONS: Nephron sparing surgery appears safe for patients with unilateral Wilms tumor associated with predisposing syndrome(s), allowing for the preservation of renal function and good oncologic outcomes for the available followup time. If more studies confirm our observation, current recommendations for the surgical treatment of Wilms tumor may need to reemphasize the value of attempting nephron sparing surgery in this patient population.

Concurrent Multicystic Dysplastic Kidney, Posterior Urethral Valves, and Obstructive Ureterocele in a Male Pediatric Patient: A Case Report.

Congenital anomalies of the kidney and urinary tract (CAKUT) are diagnosed in approximately 3-6 per 1000 live births and represent a spectrum of urologic conditions impacting the kidneys, ureter, bladder, and urethra.1 Although both are considered under the classification of CAKUT, there is no known unifying pathophysiologic mechanism for ureteroceles and posterior urethral valves with only 1 case report noted in the literature. Herein we report the only documented case of a patient with CAKUT related to posterior urethral valves, ureterocele, and multicystic dysplastic kidney.