Kidney Function and Body Composition in Adult Survivors of Unilateral, Non-Syndromic Wilms Tumor: A Report From the St. Jude Lifetime Cohort Study.

Background: The relationships among treatment exposures, body composition, and estimated glomerular filtration rate (eGFR) in adult survivors of Wilms tumor have not been well studied.

Methods: We evaluated body composition with dual-energy x-ray absorptiometry (DXA) and eGFR with the updated Chronic Kidney Disease Epidemiology Collaboration equations (creatinine only-eGFR, cystatin C only-eGFR, creatinine and cystatin C-eGFR) without race in 134 adults previously treated for unilateral, non-syndromic Wilms tumor at St. Jude Children's Research Hospital between 1964 and 2004 with chemotherapy and with (hemiabdomen [HA] or whole abdomen [WA]) or without radiation therapy (RT).

The accuracy of estimating equations for the evaluation of kidney function in adult survivors of unilateral, nonmetastatic, non-syndromic Wilms tumor: A pilot study from the St. Jude Lifetime Cohort Study.

Background: Adult survivors of unilateral, nonmetastatic, non-syndromic Wilms tumor (WT) treated with whole abdomen radiation therapy (WART) are at risk for impaired kidney function. The impact of bias and accuracy on estimated glomerular filtration rate (eGFR) among adult survivors of WT has not been well documented.

Procedure: We clinically evaluated male and female WT survivors with creatinine and cystatin C, calculated eGFR using the Chronic Kidney Disease-Epidemiology equations with and without cystatin C, and measured Tc diethylenetriamine pentaacetic acid (DTPA) plasma clearance.

Integrating Audiovisual Immersion Into Pediatric Radiation Therapy Across Multiple Centers: Methodology, Timeliness, and Cost of the Audiovisual-Assisted Therapeutic Ambience in Radiation Therapy Prospective Multi-Institutional Trial.

Purpose: The Audiovisual-Assisted Therapeutic Ambience in Radiotherapy (AVATAR) trial was a prospective multicenter study (NCT03991156) examining the combination of video immersion with radiation therapy and was successfully conducted through the collaboration of pediatric radiation oncology teams at 10 institutions independent of any pre-existing consortium. We sought to analyze and report the methodology of trial conception and development, process map, and cost.

Methods And Materials: The study enrolled patients aged 3 to 10 years preparing to undergo radiation therapy, integrated the combination of AVATAR-based video immersion with radiation therapy at each institution, and offered AVATAR use as an alternative to anesthesia, with rates of anesthesia use and outcomes of serial standardized anxiety and quality-of-life assessments assessed among the 81 children enrolled.

Wilms Tumor with Vena Caval Intravascular Extension: A Surgical Perspective.

Wilms tumor (WT) is the most common kidney tumor in pediatric patients. Intravascular extension of WT above the level of the renal veins is a rare manifestation that complicates surgical management. Patients with intravascular extension are frequently asymptomatic at diagnosis, and tumor thrombus extension is usually diagnosed by imaging.

Utilization of Palliative Radiation in Pediatric Oncology Patients During the End-of-Life (EOL).

Background: Suffering at the end-of-life (EOL) can impact the perception of a "good death" and ultimately affect bereavement for families of children with cancer. Palliative radiation (pXRT) is a tool that can address pain, mitigate suffering and improve quality of life.

Methods: A retrospective medical record review of pediatric oncology patients who died over an 11-year period was completed.

Concomitant Wilms tumor and autosomal dominant polycystic kidney disease.

Background: Concomitant Wilms tumor (WT) and autosomal dominant polycystic kidney disease (ADPKD) is exceedingly rare, presenting a diagnostic and technical challenge to pediatric surgical oncologists. The simultaneous workup and management of these disease processes are incompletely described.

Procedure: We performed a retrospective analysis of patients treated at our institution with concomitant diagnoses of WT and ADPKD.

Response of bilateral Wilms tumor to chemotherapy suggests histologic subtype and guides treatment.

Background: Patients with bilateral Wilms tumor initially receive neoadjuvant chemotherapy to shrink the tumors and increase the likelihood of successful nephron-sparing surgery. Biopsy of poorly responding tumors is often done to better understand therapy resistance. The purpose of this retrospective, single-institution study was to determine whether initial chemotherapy response is associated with tumor histology, potentially obviating the need for biopsy or change in chemotherapy.

Evaluating the Impact of Bowel Gas Variations for Wilms' Tumor in Pediatric Proton Therapy.

(1) Background: Proton therapy, a precise form of radiation treatment, can be significantly affected by variations in bowel content. The purpose was to identify the most beneficial gantry angles that minimize deviations from the treatment plan quality, thus enhancing the safety and efficacy of proton therapy for Wilms' tumor patients. (2) Methods: Thirteen patients with Wilms' tumor, enrolled in the SJWT21 clinical trial, underwent proton therapy.

Neurocognitive impairment associated with chronic morbidity in long-term survivors of Hodgkin Lymphoma.

Thoracic radiation is associated with significant cardiopulmonary morbidities in survivors of long-term Hodgkin lymphoma and may affect neurocognitive outcomes. Survivors (N = 204; 52.5% female; mean [standard deviation] age, 36.

Monitoring of Interfractional Proton Range Verification and Dosimetric Impact Based on Daily CBCT for Pediatric Patients with Pelvic Tumors.

(1) Background: Synthetic CT images of the pelvis were generated from daily CBCT images to monitor changes in water equivalent path length (WEPL) and determine the dosimetric impact of anatomy changes along the proton beam's path; (2) Methods: Ten pediatric patients with pelvic tumors treated using proton therapy with daily CBCT were included. The original planning CT was deformed to the same-day CBCT to generate synthetic CT images for WEPL comparison and dosimetric evaluation; (3) Results: WEPL changes of 20 proton fields at the distal edge of the CTV ranged from 0.1 to 12 mm with a median of 2.

Progressive metastatic infantile fibrosarcoma with multiple acquired mutations.

Infantile fibrosarcoma is the most common soft-tissue sarcoma in children under the age of 1 yr and is defined molecularly by fusion proteins. This tumor is known to be locally invasive; however, although rare, metastases can occur. The fusion acts as a driver for tumor formation, which can be targeted by first- and second-generation inhibitors.

Adaptive Proton Therapy for Pediatric Parameningeal Rhabdomyosarcoma: On-Treatment Anatomic Changes and Timing to Replanning.

Purpose: To characterize on-treatment changes in GTV morphology in children with parameningeal rhabdomyosarcoma receiving upfront proton therapy with concurrent chemotherapy and thereby provide guidance on the timing of on-treatment imaging and adaptive replanning.

Methods And Materials: GTV was delineated on 86 simulation and weekly MR images of 15 prospectively enrolled patients (aged 1-21 years). Temporal changes from baseline in volume and surface (95% Hausdorff distance) were analyzed in relation to the need for plan verification and the resultant doses with hypothetical no treatment adaptation.

Clinical Assessment of Late Health Outcomes in Survivors of Wilms Tumor.

Objectives: We aimed to clinically characterize the health, neurocognitive, and physical function outcomes of curative treatment of Wilms tumor.

Methods: Survivors of Wilms tumor (n = 280) participating in the St. Jude Lifetime Cohort, a retrospective study with prospective follow-up of individuals treated for childhood cancer at St.

Dosimetric Advantages of Silicone-Filled Vaginal Spacers in Pediatric Proton Therapy.

We introduce a custom-made silicone-filled vaginal spacer for use during treatment of female patients receiving pelvic proton radiation therapy. Commercially available vaginal dilators can be purchased as hollow objects; when filled with a media, they can act as a beam stopper and/or tissue spacer while pushing uninvolved vaginal wall away from a high-dose region. Dosimetric advantages of these specifically constructed silicone-filled vaginal spacers were investigated when compared to the unaltered commercially available product or no vaginal spacer in pediatric proton therapy.

Leveraging Therapy-Specific Polygenic Risk Scores to Predict Restrictive Lung Defects in Childhood Cancer Survivors.

Unlabelled: Therapy-related pulmonary complications are among the leading causes of morbidity among long-term survivors of childhood cancer. Restrictive ventilatory defects (RVD) are prevalent, with risks increasing after exposures to chest radiotherapy and radiomimetic chemotherapies. Using whole-genome sequencing data from 1,728 childhood cancer survivors in the St.

The myogenesis program drives clonal selection and drug resistance in rhabdomyosarcoma.

Rhabdomyosarcoma (RMS) is a pediatric cancer with features of skeletal muscle; patients with unresectable or metastatic RMS fare poorly due to high rates of disease recurrence. Here, we use single-cell and single-nucleus RNA sequencing to show that RMS tumors recapitulate the spectrum of embryonal myogenesis. Using matched patient samples from a clinical trial and orthotopic patient-derived xenografts (O-PDXs), we show that chemotherapy eliminates the most proliferative component with features of myoblasts within embryonal RMS; after treatment, the immature population with features of paraxial mesoderm expands to reconstitute the developmental hierarchy of the original tumor.

Risk factors associated with metastatic site failure in patients with high-risk neuroblastoma.

Purpose: This retrospective study sought to identify predictors of metastatic site failure (MSF) at new and/or original (present at diagnosis) sites in high-risk neuroblastoma patients.

Methods And Materials: Seventy-six high-risk neuroblastoma patients treated on four institutional prospective trials from 1997 to 2014 with induction chemotherapy, surgery, myeloablative chemotherapy, stem-cell rescue, and were eligible for consolidative primary and metastatic site (MS) radiotherapy were eligible for study inclusion. Computed-tomography and I-123 MIBG scans were used to assess disease response and Curie scores at diagnosis, post-induction, post-transplant, and treatment failure.

Revised clinical and molecular risk strata define the incidence and pattern of failure in medulloblastoma following risk-adapted radiotherapy and dose-intensive chemotherapy: results from a phase III multi-institutional study.

Background: We characterize the patterns of progression across medulloblastoma (MB) clinical risk and molecular subgroups from SJMB03, a Phase III clinical trial.

Methods: One hundred and fifty-five pediatric patients with newly diagnosed MB were treated on a prospective, multi-center phase III trial of adjuvant radiotherapy (RT) and dose-intense chemotherapy with autologous stem cell transplant. Craniospinal radiotherapy to 23.

Improved Outcome in Children With Newly Diagnosed High-Risk Neuroblastoma Treated With Chemoimmunotherapy: Updated Results of a Phase II Study Using hu14.18K322A.

Purpose: We evaluated whether combining a humanized antidisialoganglioside monoclonal antibody (hu14.18K322A) throughout therapy improves early response and outcomes in children with newly diagnosed high-risk neuroblastoma.

Patients And Methods: We conducted a prospective, single-arm, three-stage, phase II clinical trial.

Primary hypothyroidism in childhood cancer survivors: Prevalence, risk factors, and long-term consequences.

Background: Data on primary hypothyroidism and its long-term impact on the health, cognition, and quality of life (QOL) of childhood cancer survivors are limited. This study examined the prevalence of and risk factors for primary hypothyroidism and its associations with physical, neurocognitive, and psychosocial outcomes.

Methods: This was a retrospective study with a cross-sectional health outcome analysis of an established cohort comprising 2965 survivors of childhood cancer (52.