Neuroblastoma in a Newborn Female.

Neuroblastoma is the most common solid tumor in neonates. Although often aggressive in older children, carrying high mortality rates despite multimodal therapies, neuroblastoma appears to behave differently in the neonatal population. When diagnosis is clear, the disease can often be managed with close observation alone. This is a case of neuroblastoma in a 4-day-old female managed with surgical resection. This case highlights the potential challenges of diagnosis of retroperitoneal masses on prenatal ultrasound and in newborns and the importance of utilizing available resources when making difficult decisions in management.

An evaluation of onobotulinumtoxinA as a therapeutic option for pediatric neurogenic detrusor overactivity.

INTRODUCTION: Neurogenic detrusor overactivity (NDO) results in involuntary detrusor contractions during bladder filling or storage risking transmission of pressure to the upper urinary tracts and/or significant incontinence. The goals of bladder management in children with NDO prioritize the preservation of renal function, prevention of UTIs, and optimizing quality of life. First-line measures include intermittent catheterization and anticholinergic medication. However, when conservative measures fail, surgical intervention may be indicated. Historically, the next step was major reconstructive surgery to create a low-pressure urinary reservoir. The introduction of intravesical botulinum neurotoxin A (BoNT/A) for use in children in 2002 offered a less invasive option for management. However, its exact role is still evolving. AREAS COVERED: This article summarizes the mechanism of action of BoNT/A for management of NDO and evaluates the current literature defining common practice and clinical efficacy in children with NDO. The findings of the recently completed phase III trial for intravesical onabotulinumtoxinA in children are discussed in detail. EXPERT OPINION: As the first BoNT/A approved for use in children with NDO, onabotulinumtoxinA appears to be a safe and less invasive alternative to major reconstructive surgery. However, data defining appropriate patient selection and its role as a long-term treatment option continue to develop.

Understanding factors influencing primary treatment with intradetrusor onabotulinumtoxinA versus augmentation cystoplasty in patients with spina bifida.

PURPOSE: Surgical interventions in the urologic management of children with neurogenic bladder secondary to spina bifida aim to preserve upper tract function, prevent urinary tract infections, and optimize quality of life. However, since the introduction of intravesical onabotulinumtoxinA (Botox) in the management of these patients, the indications for choosing Botox over augmentation cystoplasty (AC) remain undefined. The objective of this study was to determine which factors lead patients to undergo Botox versus AC as a primary surgical treatment after failing medical management. METHODS: We retrospectively reviewed the records of pediatric patients with myelomeningocele undergoing either primary Botox or primary AC at our institution between 2013 and 2018. We recorded demographic and clinical information. We identified 10 important clinical decision-making factors: bladder trabeculation, vesicoureteral reflux, or hydronephrosis on imaging; end-filling pressure (EFP) ≥40 cm H2O, detrusor overactivity, detrusor-sphincter dyssynergia, or reduced capacity on urodynamic studies; physician-perceived bladder hostility; and patient/family desire for continence and independence. The presence of these factors was compared between patients undergoing either primary Botox or primary AC. RESULTS: We identified 14 and 50 myelomeningocele patients who underwent primary AC and primary Botox, respectively. We found no significant differences in age, sex, race, or history of reconstructive surgery (antegrade continence enema or catheterizable channel). For the 10 decision-making factors, desire for independence/continence (p = <0.001) and reduced capacity (p = 0.002) were significantly associated with AC, while trabeculation (p = 0.006), EFP ≥40 cm H2O (p = 0.029), rising slope (p = 0.019), and physician-perceived hostility (p = 0.012) were significantly more common with Botox. CONCLUSIONS: At our institution, quality of life measures prompted AC over objective urodynamic or imaging findings before attempting Botox. These findings support a shared decision-making approach when considering surgical intervention for neurogenic bladder secondary to myelomeningocele.

Gonadal tumors in a contemporary cohort of patients with differences in sex development undergoing surgery - A multi-site study from the Pediatric Urologic Oncology Working Group of the societies for pediatric urology.

BACKGROUND: Disturbances in gonadal development lead to increased risk of gonadal malignancy in some but not all patients with differences in sex development (DSD). However, the natural history of these tumors is poorly described, and the literature remains sparse. OBJECTIVE: The objective of this study was to describe the incidence of germ cell neoplasia in situ (GCNIS) and germ cell tumor (GCT) in a contemporary cohort of patients with DSD undergoing surgery and to provide long-term oncologic outcomes for these patients. STUDY DESIGN: Patients with DSD who have undergone gonadectomy or gonadal biopsy were identified at four institutions. Clinical characteristics, pathology, and treatment details were obtained retrospectively. Patients were stratified into risk categories based on DSD diagnosis. Oncologic treatment and outcomes were recorded. Descriptive statistics are reported using parametric methods. RESULTS: 83 patients were identified. Distribution of diagnoses is summarized in the summary table. 14 (16.9%) patients underwent gonadal biopsy, and 71 (85.5%) patients underwent gonadectomy (50/71 gonadectomies were bilateral). 8/83 (9.6%) patients had GCNIS or GCT (7 GCNIS, 1 GCT). Median age at surgery was 2.95 years (y) (interquartile range [IQR] 0.6-12.2) and 14y (IQR 0.85-16.9) in patients without and with GCNIS/GCT, respectively. All 8 patients with GCNIS/GCT had high or intermediate risk DSD diagnoses (4 mixed gonadal dysgenesis, 3 Turner with Y, 1 partial gonadal dysgenesis). Of the patients with high-risk diagnoses, 8/54 (15%) had GCNIS/GCT. No patient received adjuvant therapy, no patient had a recurrence, and all patients were living with mean follow up 6.4y. DISCUSSION: The risk of gonadal malignancy is heterogeneous in the DSD population and can vary based on DSD diagnosis as well as maturation, testicularization, and location of the gonads. The most recent consensus recommendations on gonadal management emphasize risk stratification and consideration of gonadal surveillance based on gender of rearing, but supporting literature remains sparse. In this contemporary cohort of DSD patients who underwent gonadal surgery, most patients did not have evidence of adverse pathology, all patients with malignant or premalignant pathology had a high/intermediate risk DSD diagnosis, and all patients with GCNIS/GCT were treated with surgery alone without recurrence. CONCLUSIONS: The distribution of patients with premalignant and malignant gonadal pathology and DSD in this cohort aligns with prior literature, and oncologic outcomes were excellent. These data add valuable information to the current literature and highlight the necessity to develop appropriate screening regimens for retained gonads.

Investigation of urology intraoperative events leading to root cause analysis at national VA medical centers.

INTRODUCTION: Adverse events in urologic procedures are poorly studied. This study analyzes the Veterans Health Administration (VHA) Root Cause Analysis (RCA) data for patient safety adverse events during urologic procedures performed in a VHA operating room (OR). MATERIALS AND METHODS: The VHA National Center for Patient Safety RCA database was queried for fiscal years 2015-2019 using urologic terms including vasectomy, prostatectomy, nephrectomy, cystectomy, cystoscopy, lithotripsy, ureteroscopy, urethral, TURBT, etc. RCAs for events outside a VHA OR were excluded. Cases were categorized based on type of event. RESULTS: Sixty-eight RCAs were identified for 319,713 urologic procedures. The most common pattern identified was equipment or instrument issue, including broken scopes or smoking light cords, with 22 cases. Eighteen RCAs involved a sentinel event, including 12 retained surgical items (RSI) (surgical sponge, retained guidewire) and 6 wrong site surgeries (WSS) (incorrect laterality, wrong procedure) representing a serious safety event rate of 1 in 17,762 procedures. In addition, 8 RCAs pertained to medical or anesthesia events (incorrect dosing, postoperative myocardial infarction), 7 to pathology errors (missing or mislabeled specimen), 4 to incorrect patient information or consent, and 4 to surgical complications (hemorrhage, duodenal injury). In 2 cases there was inappropriate work up. One case caused a delay in treatment, one case had an incorrect count, and one case identified lack of credentialing. CONCLUSIONS: RCAs of patient safety adverse events occurring during urologic OR procedures highlight the need for targeted quality improvement projects to prevent WSS events, prevent RSI events, and maintain properly functioning equipment.

Bilateral Wilms Tumor in CLOVES Syndrome.

Wilms tumor is the most common pediatric renal mass and occurs in up to 10% of predisposition syndromes. One such syndrome is CLOVES syndrome, an extremely rare disorder within the umbrella of PIK3CA-related overgrowth spectrum disorders. This case presents the management of a bilateral Wilms tumor in a patient with CLOVES syndrome and highlights the many intricacies in caring for complex oncology patients. Particularly highlighted in this case is the delicate line in balancing the risks of treatment-related morbidity against the risks of recurrence in predisposed patients, while still abiding by established treatment guidelines.

Ectopic Vas Deferens in a Male Infant.

Vas deferens ectopia is a rare congenital anomaly associated with urinary tract and anorectal anomalies. It is important to recognize the complex embryology of the reproductive, urinary, and lower enteric systems to understand how these processes can go awry.1 As the ureteric bud arises from the mesonephric duct, a failure of the channel that connects the two to obliterate can produce an ectopic vas deferens connected to the ureter or bladder.2 We present a 2-month-old male with vas deferens ectopia. Our patient uniquely did not present with associated anorectal anomalies or urinary tract symptoms.

Validation of the modified Bosniak classification system to risk stratify pediatric cystic renal masses: An international, multi-site study from the pediatric urologic oncology working group of the societies for pediatric urology.

BACKGROUND: Pediatric cystic renal lesions are challenging to manage as little is known about their natural course. A modified Bosniak (mBosniak) classification system has been proposed for risk stratification in pediatric patients that takes ultrasound (US) and/or computed tomogram (CT) characteristics into account. However, literature validating this system remains limited. OBJECTIVE: To determine if the mBosniak classification system correlates with pathologic diagnoses. The hypothesis is that mBosniak classification can stratify the risk of malignancy in children with renal cysts. STUDY DESIGN: Patients treated for cystic renal masses with available imaging and pathology between 2000 and 2019 from five institutions were identified. Clinical characteristics and pathology were obtained retrospectively. Characteristics from the most recent US, CT, and/or magnetic resonance imaging (MRI) were recorded. Reviewers assigned a mBosniak classification to each scan. mBosniak scores 1/2 were considered low-risk and 3/4 high-risk. These groups were compared with pathology (classified as benign, intermediate, malignant). Sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), positive likelihood ratio (+LR), and negative likelihood ratio (-LR) were calculated to assess this categorization as a screening tool to guide surgical intervention. Agreement between imaging modalities was also explored. RESULTS: 99 patients were identified. High-risk imaging findings were correlated with malignant or intermediate pathology with a sensitivity of 88.3%, specificity of 84.6%, PPV of 89.8%, NPV of 82.5%, +LR of 5.7, and -LR of 0.14. The sensitivity for detecting malignant lesions only was 100%. There was substantial agreement between US/CT (n = 55; κ = 0.66) and moderate agreement between US/MRI (n = 20; κ = 0.52) and CT/MRI (n = 13; κ = 0.47). DISCUSSION: The mBos classification system is a useful tool in predicting the likelihood of benign vs. intermediate or malignant pathology. The relatively high sensitivity and specificity of the system for prediction of high-risk lesions makes this classification applicable to clinical decision making. In addition, all malignant lesions were accurately identified as mBosniak 4 on imaging. This study adds substantial data to the relatively small body of literature validating the mBosniak system for risk stratifying pediatric cystic renal lesions. CONCLUSIONS: Pediatric cystic renal lesions assigned mBosniak class 1/2 are mostly benign, whereas class 3/4 lesions are likely intermediate or malignant pathology. We observed that the mBosniak system correctly identified pathology appropriate for surgical management in 88% of cases and did not miss malignant pathologies. There is substantial agreement between CT and US scans concerning mBos classification.

Gonadal malignancy in patients with differences of sex development.

Differences of sexual development (DSD) are known to be associated with an elevated risk of malignant and pre-malignant tumors. However, given the rarity of DSD and tumors in patients with DSD, more robust, large scale, prospective literature is required to truly determine the extent of this association, long-term outcomes and the nuances associated with the wide variety of DSD diagnoses. In addition, the spectrum of diagnoses and nomenclature has been ever-changing, limiting assessment of long-term patient outcomes. This review aims to provide an overview of the pathogenesis of DSD conditions, potential malignancies associated with the diagnoses, the available screening for malignancy, and the most recent data on stratification for each DSD diagnosis and association with malignancy.

Oncologic outcomes of pre-malignant and invasive germ cell tumors in patients with differences in sex development - A systematic review.

OBJECTIVE: To describe the rates of GCNIS-free and GCT-free pathology based on age at gonadal surgery and to describe long-term oncologic outcomes in patients with DSD who have GCNIS or GCT at the time of gonadal surgery. STUDY DESIGN: A systematic review was conducted using MEDLINE to identify patients with DSD who underwent gonadal surgery. DSD diagnoses were stratified based on malignancy risk. GCNIS/GCT and GCT-free survival by age of gonadal surgery, RFS and OS were calculated using the Kaplan-Meier method, with groups compared using log-rank testing. RESULTS: 386 articles from 1951 to 2017 were included (2037 patients). Median age at gonadal surgery was 17 years (y) (IQR 11-20), median follow-up was 60 months (m) (IQR 30-68.1). GCNIS/GCT- and GCT-free survival at the time of gonadal surgery was lowest for those in the high/intermediate risk group (p < 0.001) but decreased sharply around age 15y, regardless of risk category. 5y RFS and OS was similar for those with no GCNIS/GCT and GCNIS and was worse for those with GCT (p < 0.001). DISCUSSION: When patients undergo gonadal surgery, regardless of indication (i.e. prophylactic vs. tumor), it appears that GCTs are more commonly found when surgery is done around age 15 y or older, despite risk category. This is similar to ovarian and testicular GCTs. Patients with GCNIS can be reassured that long-term oncologic outcomes are excellent. While RFS and OS for GCTs are not as good as for ovarian and testicular GCTs (95%), they are still >80%. This similar trend was found in a COG review of 9 patients with DSD and ovarian GCT. There were several limitations to this study. This is a retrospective analysis that included aa wide time frame of publications. The indication for surgical intervention was not addressed in the majority of publications. Thus these data provide pathologic outcomes based on age at gonadal surgery rather than the age at which GCNIS/GCT develops over a lifetime, if at all. CONCLUSIONS: The risk of GCNIS or GCT at the time of gonadal surgery appears to increase with age, accelerating between 15 and 20y regardless of risk category. 5y RFS and OS for those with GCNIS is equivalent to those without GCNIS/GCT but is worse for those with GCT. These data may be used when counseling families on timing of gonadal surgery and quantification of outcomes should GCNIS or malignancy be identified.

Recurrent Bladder Cancer in a Teenage Male.

Urothelial cell carcinoma of the bladder is exceedingly rare in pediatric patients. Based on current literature, bladder cancer in this population is thought to be low-grade, noninvasive, and unlikely to recur, suggesting significant biologic differences when compared to the adult population. This is a 15-year-old male diagnosed with low-grade urothelial cell carcinoma with subsequent multifocal recurrence on surveillance cystoscopy managed by induction and maintenance intravesical Bacillus Calmette-Guerin. This case highlights the challenges of management and surveillance of this rare disease.

International Publication Patterns on Operative Management of Wilms Tumor: A Review by Surgeon Specialty and Surgical Modality.

OBJECTIVE: To review the temporal trends in the published literature of the surgical management of Wilms tumor (WT) relative to surgical modality and authorship by surgeon specialty (pediatric urology [PU] vs pediatric surgery [PS]). METHODS: The MEDLINE/PubMed database was queried for manuscripts published by PU or PS including operative management of WT from 1993-2017. After manuscript review, 187 of 2420 studies were included. Studies were compared by author specialty, country of origin, and represented patients. RESULTS: PU published 32% of included publications. The proportion of first author publications by PU increased over time (25% from 1993-2005 to 39% from 2006-2017, P = .029). While PU were first author on 44% of studies in North America, they were relatively underrepresented internationally. PU and PS were equally likely to be first author on cooperative group studies, which accounted for 19% of the overall publications. 14,958 patients underwent extirpative surgery for WT. There was a small increase in publications reporting the use of minimally-invasive surgery (MIS) and nephron-sparing surgery (NSS) over time (<1% and 4% from 1993-2005 to 2% and 7% from 2006-2017, respectively, P <.001). Compared to PS, PU were significantly more likely to publish on patients managed with MIS and NSS (P = .006 and P < 0.001 respectively). CONCLUSION: Publications by PU on the surgical management of WT are increasing over time, but are still fewer than PS. PU appeared to be the drivers of the temporal trend towards the increasing use of MIS and NSS in the published literature.

Open Radical Nephrectomy for Suspected Renal Malignancy-Tips and Tricks.

OBJECTIVE: To present the surgical approach to a cystic renal mass suspicious for malignancy in a 22-month-old female. MATERIALS: The patient was a healthy female was found to have an abdominal mass by her parents. Her mother underwent a nephrectomy as a child for unknown pathology. Physical examination was otherwise unremarkable and laboratory workup was normal. Imaging workup revealed a large cystic renal mass suspicious for malignancy. Surgical resection was planned. RESULTS: A nonsyndromic 22-month-old female underwent open radical nephrectomy on the left with regional lymph node dissection. A transverse abdominal incision was used, followed by medial reflection of the colon and complete renal mobilization. The distal ureter and renal vasculature were identified and controlled and the kidney removed. A retractor was then assembled and regional lymph node dissection performed. Final pathology was cystic nephroma with 10 benign lymph nodes. The patient recovered well and was discharged home 3 days after surgery. CONCLUSION: Open radical nephrectomy is an important part of protocol management for patients with renal masses suspicious for malignancy.

Pediatric pheochromocytoma: current status of diagnostic imaging and treatment procedures.

PURPOSE OF REVIEW: To provide an overview of relevant data available and updated recommendations for management of pediatric patients with pheochromocytoma (PCC). RECENT FINDINGS: Much of the available data surrounding pediatric PCC is in the form of case reports and case series. With the accumulation of data over time, pediatric PCC does in fact differ significantly from not only what is known in the adult population, but also from classic teaching. Pediatric patients are much more likely to have a hereditary predisposition as well as aggressive and malignant disease. Much of the recent literature focuses on defining these genetic syndromes in order to provide recommendations for screening and genetic counseling. Other recent advances center around developing treatments for metastatic disease. Timely diagnosis with plasma metanephrines and cross-sectional imaging, and appropriate preoperative medical optimization followed by surgical resection remain the center of treatment. SUMMARY: Although rare and adult principles are applied to pediatric PCC, genetic testing plays a pivotal role in management of children, adolescents and young adults with PCC.

Examining and Understanding Value: The Impact of Preoperative Characteristics, Intraoperative Variables, and Postoperative Complications on Cost of Robot-Assisted Laparoscopic Radical Prostatectomy.

Introduction and Objective: Quality-based reimbursement continues to gain popularity as value-based care becomes more prominent. Our goal is to describe the impact of preoperative characteristics, intraoperative variables, and postoperative complications on the cost of robot-assisted laparoscopic radical prostatectomy (RALP). Materials and Methods: Using our institution's National Surgical Quality Improvement Program (NSQIP) data, we identified minimally invasive prostatectomies performed from January 2012 to March 2017. A retrospective chart review was done to collect perioperative data; financial data were collected from the business office. Results: Two hundred seventy-five patients were identified during this time period. Median total cost was $16,600 (interquartile range $15,100-$18,300), and median direct cost (DC) was $11,200 ($10,100-$12,400). Among preoperative characteristics, body mass index (BMI) ≥30 kg/m2, diabetes, hypertension, and blood urea nitrogen >21 were associated with increased DCs of $500, $500, $200, and $600, respectively (p < 0.05). American Society of Anesthesiologists (ASA) class III was associated with increased DC of $200 compared with ASA classes I-II (p < 0.05). Considering intraoperative characteristics, increasing operative times and estimated blood loss (EBL) were associated with increased DC (p < 0.001, p < 0.05, respectively). Occurrence of any postoperative complication was associated with increased DC of $1400 (p < 0.05). On multivariable analysis, a 1-U increase in BMI was associated with a $129 increase in DC (p < 0.001), a length of stay (LOS) greater than 3 days was associated with a $4099 increase in DC (p < 0.001), a 30-minute increase in operating room duration was associated with a $410 increase in DC (p < 0.05), any postoperative complication was associated with a $5397 increase in DC (p < 0.01), and treatment for diabetes was associated with a $1860 increase in DC (p < 0.05). Conclusion: BMI, diabetes, operative duration, EBL, LOS, and postoperative complications were associated with significantly increased DC of RALP. Understanding perioperative factors affecting cost contributes to understanding value in prostatectomy and improving quality in urologic care.

Dermatofibrosarcoma Protuberans in a Male Infant.

Dermtofibrosarcoma protuberans is a rare cutaneous malignancy known to be locally aggressive. It is uncommonly seen in the pediatric population and can be difficult to distinguish from other benign skin lesions. We present a case of dermatofibrosarcoma protuberans of the penis in a 6-month-old child managed with surgical resection. This case highlights the challenges of diagnosis of genital lesions in children and the complexities of genitourinary reconstruction following surgical resection.

Collision metastasis of prostatic adenocarcinoma and urothelial carcinoma of the bladder.

The incidence of concomitant prostate adenocarcinoma found in patients with muscle-invasive bladder carcinoma is not uncommon, reaching up to 21%-28%. However, the presence of collision metastasis involving prostate cancer and bladder cancer within the same lymph node is exceedingly rare, with only 5 cases reported to date in the literature. We report a case of collision metastasis of prostate adenocarcinoma and urothelial carcinoma of the bladder in a 73-year-old man who underwent cystoprostatectomy with bilateral pelvic lymph node dissection for high-grade muscle-invasive urothelial carcinoma. Final pathology revealed a pT3aN2 high-grade urothelial carcinoma and pT3N1 Gleason 4 + 4 = 8 adenocarcinoma of the prostate with 12/40 pelvic lymph nodes positive for urothelial carcinoma. One node was positive for both urothelial carcinoma and prostate adenocarcinoma, confirmed by positive staining by p40 and prostate specific antigen(PSA), respectively. Immunohistochemistry is the sole method of confirming collision metastasis of two primary cancers. In this case, we describe immunohistochemical markers for urothelial carcinoma and prostate adenocarcinoma and their clinical implications. One month postoperatively, our patient began adjuvant leuprolide therapy and cycle 1 of gemcitabine and cisplatin chemotherapy, which he is tolerating well.

Continent Catheterizable Vesicostomy: An Alternative Surgical Modality for Pediatric Patients With Large Bladder Capacity.

OBJECTIVE: To present a modified technique and early outcomes of a continent catheterizable vesicostomy in pediatric patients with either flaccid neurogenic bladder or intractable voiding dysfunction and large capacity bladder. METHODS: Six patients underwent the procedure from October 2014 to December 2015. A 4-cm Pfannenstiel incision was made, avoiding intraperitoneal dissection. After adequate mobilization, a 2-cm vertical flap at the dome of the bladder was identified and tubularized over a 12Fr catheter with 4-0 vicryl suture. The tubularized flap was then intussuscepted into the bladder with four 4-0 polydioxanone sutures, creating a continent mechanism. The catheterizable channel was then tunneled to the umbilicus, the channel ostomy matured, and the cystotomy closed in two layers. RESULTS: The median patient age was 8 (interquartile range [IQR] 12) years. All patients had urinary dysfunction requiring drainage from etiologies that included Eagle-Barrett syndrome (n = 2), Noonan syndrome (n = 1), Lennox-Gastaut syndrome (n = 1), and Spina bifida (n = 2). Median hospital length of stay was 8 (IQR 3) days. One patient had a superficial wound infection treated with antibiotics, and 1 patient required balloon dilation of the catheterizable channel at 3 months postoperatively, secondary to difficulty self-catheterizing. Five patients were successfully self-catheterizing at last follow-up. Median follow-up was 6 (IQR 5) months and there were no intra- or perioperative complications. CONCLUSION: Continent catheterizable vesicostomy is a novel technique for urinary drainage in patients with large bladder capacity that spares use of the appendix or ileum. Early results are encouraging, providing a catheterizable channel through the umbilicus without urinary leakage between catheterization.