Extended Lymph Node Sampling During Surgery for Pediatric Renal Tumors Concerning for Malignancy Does Not Increase Postoperative Complication Rates.

PURPOSE: Although Children's Oncology Group renal tumor protocols mandate lymph node sampling during extirpative surgery for pediatric renal tumors, lymph node sampling is often omitted or low yield. Concerns over morbidity associated with extended lymph node sampling have led to hesitancy in adopting a formal lymph node sampling template. We hypothesized that complications in children undergoing lymph node sampling for renal tumors would be rare, and not associated with the number of lymph nodes sampled. MATERIALS AND METHODS: A single-institution, retrospective review of patients aged 0-18 years undergoing extirpative renal surgery with lymph node sampling for a suspected malignancy between 2005 and 2019 was performed. Patients with 0 or an unknown number of lymph nodes sampled or <150 days of follow-up were excluded. A "clinically significant" complication was defined as any Clavien complication ≥III, small-bowel obstruction, chylous ascites, organ injury, or wound infection. The number of lymph nodes sampled and its influence on the odds of experiencing a clinically significant complication was examined. RESULTS: A total of 144 patients met inclusion criteria. Median patient age was 38 months. Twenty-one patients (15%) had a clinically significant complication, the most common of which was ileus/small-bowel obstruction (n=16). In a multivariable analysis, increased lymph node yield was not found to influence the odds of experiencing a clinically significant complication (P = .6). CONCLUSIONS: In this cohort, there was no statistically significant difference in clinically significant complications in patients who underwent more extensive lymph node sampling during surgery for a suspected malignant pediatric renal tumor. Future studies on protocol adherence, staging accuracy, and survival trends using a lymph node sampling template in these patients should be performed.

Pediatric Small Renal Masses: Can Tumor Size Predict Histology and the Potential for Nephron-sparing Surgery?

PURPOSE: The majority of children with unilateral renal masses suspicious for malignancy undergo radical nephrectomy, while nephron-sparing surgery is reserved for select cases. We investigated the impact of tumor size on the probability of histology. We hypothesized that pediatric small renal masses are more likely benign or non-Wilms tumor, thus potentially appropriate for nephron-sparing surgery. MATERIALS AND METHODS: The SEER (Surveillance, Epidemiology, and End Results) database was analyzed for patients aged 0-18 years diagnosed with a unilateral renal mass from 2000-2016. Statistical analysis was performed to help determine a tumor size cut point to predict Wilms tumor and assess the predictive value of tumor size on Wilms tumor histology. Additionally, a retrospective review was performed of patients 0-18 years old who underwent surgery for a unilateral renal mass at a single institution from 2005-2019. Statistical analysis was performed to assess the predictive value of tumor size on final histology. RESULTS: From the SEER analysis, 2,016 patients were included. A total of 1,672 tumors (82.9%) were Wilms tumor. Analysis revealed 4 cm to be a suitable cut point to distinguish non-Wilms tumor. Tumors ≥4 cm were more likely Wilms tumor (OR 2.67, P ≤ .001), but this was driven by the statistical significance in children 5-9 years old. From the institutional analysis, 134 patients were included. Ninety-seven tumors (72.3%) were Wilms tumor. Tumors ≥4 cm had higher odds of being Wilms tumor (OR 30.85, P = .001), malignant (OR 6.75, P = .005), and having radical nephrectomy-appropriate histology (OR 46.79, P < .001). CONCLUSIONS: The probability that a pediatric unilateral renal mass is Wilms tumor increases with tumor size. Four centimeters is a logical cut point to start the conversation around defining pediatric small renal masses and may help predict nephron-sparing surgery-appropriate histology.

Satisfaction and Shared Decision Making in a Multidisciplinary Care Clinic for Patients with Differences in Sex Development.

INTRODUCTION: Patients with differences in sex development represent a complex pediatric population with varying psychosocial and medical needs. Due to the complexity of care, families likely benefit from multidisciplinary care allowing for coordination of psychosocial and health services. Unfortunately, there are few data on the experiences of families of patients with differences of sex development in a multidisciplinary setting, such as their satisfaction with health care or their level of shared decision making. METHODS: Patients and guardians seen in a multidisciplinary, differences in sex development clinic were asked to participate in an anonymous online survey of their satisfaction with health care delivery (Patient Satisfaction Questionnaire Short Form) and involvement in shared decision making (Shared Decision Making Questionnaire). Welch's t-test was used to compare mean survey scores to historical and contemporary control populations. RESULTS: In all, 22 guardians and 1 young adult patient completed surveys. Median patient age was 36 months. Patient diagnoses were diverse, with the most common diagnosis beings 46, XY DSD (34.8%). At the time of their clinic visit, 7 patients had undergone surgery. Mean satisfaction scores were higher than a primary care population and comparable to patients and patients' caregivers with chronic medical conditions. The mean shared decision making score was well above adult populations and similar to caregiver reports in pediatric outpatient clinics. CONCLUSIONS: Despite the complex treatment associated with differences of sex development, families are very satisfied with the quality of care they receive in a multidisciplinary setting and appear to feel personally invested in the decision making process.

Neurofibromatosis-1 and Rhabdomyosarcoma: An Unusual Recurrence.

Neurofibromatosis-1 has a known increased risk of malignancy with rhabdomyosarcoma occurring in up to 6% of patients. Here we report on an 8-year-old male with a history of Neurofibromatosis-1 and previously treated stage 3, group III bladder/prostate embryonal rhabdomyosarcoma (diagnosed at 18 months old) who presented with penile swelling concerning for priapism. Imaging and subsequent biopsy confirmed embryonal rhabdomyosarcoma of the penile corporal bodies. Penile rhabdomyosarcoma is exceedingly rare, with less than 15 case reports in the literature. Our patient received chemoradiation per D9803 with organ preserving local control and is doing well 3 months after treatment.

Factors affecting lymph node sampling patterns and the impact on survival of lymph node density in patients with Wilms tumor: a Surveillance, Epidemiology, and End Result (SEER) database review.

BACKGROUND: Lymph node (LN) involvement is an important prognostic indicator for patients with Wilms tumor (WT), and there have been previous reports of utilizing LN density (LND = positive LN/LNs examined) as an advanced metric to risk-stratify patients with WT. OBJECTIVE: The purpose of this study was to describe patient characteristics that affect LN yield and assess the effect of LND on the overall survival (OS) in patients with WT, with the expectation that patients with LNDs above a critical cut-point would demonstrate lower OS. STUDY DESIGN: The Surveillance, Epidemiology, and End Result (SEER) database was queried for all patients diagnosed with unilateral WT from 2004 to 2015. Patient and disease characteristics were collected, and Poisson regression was used to identify characteristics correlated with LN yield. LND was calculated for LN-positive patients, and multivariable survival analysis was performed, including patient demographics and LND as variables. RESULTS: 1489 patients with unilateral WT were identified for analysis, 231 (15.51%) of whom were LN-positive. Median patient age at diagnosis was three years (IQR 1-5). On Poisson regression, the year of diagnosis, patient age, tumor size and laterality, and stage were found to impact LN yield. For patients with positive LNs, five-year OS of patients with LNDs above 0.4 was worse than those below 0.4 (76.1% vs 89.6%, p = 0.041). On multivariable analysis, tumor size and LND remained significant predictors of OS. DISCUSSION: Administrative databases such as SEER provide an excellent resource for studying conditions where large patient numbers for analysis are difficult to obtain. Unfortunately, the SEER database is unable to account for every factor that could affect LN sampling patterns. Additionally, favorable vs unfavorable histology is not available in SEER, and SEER utilizes its own staging system, which makes comparison to Children's Oncology Group staging difficult. Despite these limitations, the findings of this study are similar to those previously published using administrative databases analyzing LN sampling patterns and the effect of LND on OS in WT. CONCLUSIONS: Analysis of the SEER database confirms that there are several patient- and disease-specific factors that affect the number of LNs sampled during nephrectomy for WT, and that LND may be a predictor of OS. These findings highlight the need for standardization of LN sampling patterns for pediatric renal tumors and support the investigation of LND in future studies to further risk-stratify WT patients to tailor therapy intensity.

Isolated Pure Malignant Rhabdoid Tumor (MRT) of the Bladder: Case Report and Lessons Learned.

Pediatric extrarenal malignant rhabdoid tumors (MRTs) are rare, aggressive tumors with a poor prognosis (20% 5-year survival). There are currently fewer than 10 published case reports of primary MRT of the bladder. We report the case of an 18-month-old female with an isolated MRT of the bladder which was initially misdiagnosed as an inflammatory myofibroblastic tumor on biopsy. We review the history, tumor biology, histology, and current management of extrarenal MRT, along with lessons learned from the difficulty with the patient's initial diagnosis.

The Natural History of Wilms Tumor-A Case Comparison of Two Different Tumors.

Due to the rarity of Wilms tumor (WT) and the relative urgency with which pediatric renal tumors are treated, there is little reported data on the natural history and growth of WTs. Historical reports of estimated doubling times of WTs were based on time to disease recurrence after initial diagnosis and treatment, and were published before the current advancements in molecular biomarker testing. We compare 2 cases of WT with sequential imaging, and postulate how the growth parameters of these tumors may be associated with differing chromosomal traits.

Adult Wilms Tumor During Pregnancy: Case Report and Literature Review.

Adult Wilms tumor (WT) is a well-known, albeit rare entity and has historically been associated with worse overall clinical outcomes when compared to younger patients. Because WT is uncommon in adult patients, it is often misdiagnosed and treated off standardized pediatric protocols. WT associated with pregnancy is even more rare, and there is not a standardized approach to this small subset of patients. We present a case of an adult WT discovered and managed during the perinatal period and review prior published cases.

Open Vesicocalicostomy for the Management of Transplant Ureteral Stricture.

A 59-year-old male developed a proximal stricture of his transplant ureter ten years after a living donor renal transplant. Endoscopic management was unsuccessful, and the patient was temporized with percutaneous nephrostomy tubes for months. Eventually, it became clear he would require surgical revision. Intraoperatively, complete fibrosis of the renal hilum, and intrarenal location of the pelvis precluded the planned pyelovesicostomy. A successful open vesicocalicostomy was performed, anastomosing a bladder flap to a lower pole calix. The patient remains recurrence free after 6 months of follow-up.