Davidson, Jacob

Abstract Purpose Our objectives were to compare overall survival (OS) and pulmonary relapse between patients with metastatic Ewing sarcoma (EWS) at diagnosis who achieve rapid complete response (RCR) and those with residual pulmonary nodules after induction chemotherapy (non‐RCR). Patients and methods This retrospective cohort study included children under 20 years with metastatic EWS treated from 2007 to 2020 at 19 institutions in the Pediatric Surgical Oncology Research Collaborative. Chi‐square tests were conducted for differences among groups. Kaplan–Meier curves were generated for OS and pulmonary relapse. Results Among 148 patients with metastatic EWS at diagnosis, 61 (41.2%) achieved RCR. Five‐year OS was 71.2% for patients who achieved RCR, and 50.2% for those without RCR ( p = .04), and in multivariable regression among patients with isolated pulmonary metastases, RCR (hazards ratio [HR] 0.42; 95% confidence interval [CI]: 0.17–0.99) and whole lung irradiation (WLI) (HR 0.35; 95% CI: 0.16–0.77) were associated with improved survival. Pulmonary relapse occurred in 57 (37%) patients, including 18 (29%) in the RCR and 36 (41%) in the non‐RCR groups ( p = .14). Five‐year pulmonary relapse rates did not significantly differ based on RCR (33.0%) versus non‐RCR (47.0%, p = .13), or WLI (38.8%) versus no WLI (46.0%, p = .32). Discussion Patients with EWS who had isolated pulmonary metastases at diagnosis had improved OS if they achieved RCR and received WLI, despite having no significant differences in rates of pulmonary relapse.

Objective: To determine the impact of nodal basin ultrasound (US) surveillance versus completion lymph node dissection (CLND) in children and adolescents with sentinel lymph node (SLN) positive melanoma. Background: Treatment for children and adolescents with melanoma are extrapolated from adult trials. However, there is increasing evidence that important clinical and biological differences exist between pediatric and adult melanoma. Methods: Patients ≤18 years diagnosed with cutaneous melanoma between 2010 and 2020 from 14 pediatric hospitals were included. Data extracted included demographics, histopathology, nodal basin strategies, surveillance intervals, and survival information. Results: Of 252 patients, 90.1% (n=227) underwent SLN biopsy (SLNB), 50.9% (n=115) had at least 1 positive node. A total of 67 patients underwent CLND with 97.0% (n=65/67) performed after a positive SLNB. In contrast, 46 total patients underwent US observation of nodal basins with 78.3% (n=36/46) of these occurring after positive SLNB. Younger patients were more likely to undergo US surveillance (median age 8.5 y) than CLND (median age 11.3 y; P=0.0103). Overall, 8.9% (n=21/235) experienced disease recurrence: 6 primary, 6 nodal, and 9 distant. There was no difference in recurrence (11.1% vs 18.8%; P=0.28) or death from disease (2.2% vs 9.7%; P=0.36) for those who underwent US versus CLND, respectively. Conclusions: Children and adolescents with cutaneous melanoma frequently have nodal metastases identified by SLN. Recurrence was more common among patients with thicker primary lesions and positive SLN. No significant differences in oncologic outcomes were observed with US surveillance and CLND following the identification of a positive SLN.

Objective: The purpose of this study was to describe management and outcomes from a contemporary cohort of children with Wilms tumor complicated by inferior vena caval thrombus. Background: The largest series of these patients was published almost 2 decades ago. Since then, neoadjuvant chemotherapy has been commonly used to manage these patients, and outcomes have not been reported. Methods: Retrospective review of 19 North American centers between 2009 and 2019. Patient and disease characteristics, management, and outcomes were investigated and analyzed. Results: Of 124 patients, 81% had favorable histology (FH), and 52% were stage IV. IVC thrombus level was infrahepatic in 53 (43%), intrahepatic in 32 (26%), suprahepatic in 14 (11%), and cardiac in 24 (19%). Neoadjuvant chemotherapy using a 3-drug regimen was administered in 82% and postresection radiation in 90%. Thrombus level regression was 45% overall, with suprahepatic level showing the best response (62%). Cardiopulmonary bypass (CPB) was potentially avoided in 67%. The perioperative complication rate was significantly lower after neoadjuvant chemotherapy [(25%) vs upfront surgery (55%); P=0.005]. CPB was not associated with higher complications [CPB (50%) vs no CPB (27%); P=0.08]. Two-year event-free survival was 93% and overall survival was 96%, higher in FH cases (FH 98% vs unfavorable histology/anaplastic 82%; P=0.73). Neither incomplete resection nor viable thrombus cells affected event-free survival or overall survival. Conclusions: Multimodal therapy resulted in excellent outcomes, even with advanced-stage disease and cardiac extension. Neoadjuvant chemotherapy decreased the need for CPB to facilitate resection. Complete thrombectomy may not always be necessary.

AbstractWilms 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.

Abstract 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.

AbstractBackgroundEnteral nutrition (EN) is a safe and cost‐effective intervention with significant morbidity and mortality benefit that can also be associated with complications. Peristomal infections can occur in 5%–30% of cases after percutaneous endoscopic gastrostomy placement. Despite this common occurrence, widespread use of objective diagnostic criteria is not prevalent, possibly leading to overuse of antibiotics.MethodsAll patients who were reporting symptoms consistent with peristomal infection were evaluated. Criteria based on erythema, induration, and exudates were applied with cumulative score ≥8 indicating peristomal infection. All patients were followed, with additional factors being collected if available.ResultsForty‐three patients noted complaints consistent with peristomal infection. In patients with a score <8 (n = 16), only 2 required antibiotics (12.5%) and 1 patient was hospitalized (6.3%). In patients with a score ≥8 (n = 27), 16 had a score of 8 or 9; all patients responded to antibiotics (100%), and 1 was hospitalized (6.3%). In patients with a score ≥10, all (100%) were treated with antibiotics. Five of 11 (45%) patients were hospitalized, with the most common diagnosis being sepsis. Of the baseline variables analyzed, higher body mass index correlated with score ≥10 (26.36 ± 2.94 vs 22.91 ± 4.35 in score <9, P = .0128).ConclusionsObjective criteria were effective in risk stratifying patients who presented with reports of peristomal infection. Patients with a score <8 tended to be at low risk, scores of 8–9 were of moderate risk, and patients with a score ≥10 were at high risk and required very close monitoring or hospitalization.

AbstractBackground: Since initial report in 1972, misconnections continue to be an issue, especially in hospitalized patients with multiple access devices. A new small‐bore connector standard (ENFit) has been proposed in order to minimize misconnections. Methods: Commercially available finalized ENFit tubes of a variety of sizes (14 French [Fr] size, 18Fr, 20Fr, 24Fr, and low‐profile) were obtained for current testing. Variety of commercially available formulas including blenderized tube feeding (BTF) products (Jevity 1 Cal, Abbott Nutrition, Abbott Park, IL; Nourish, Functional Formularies, Centerville, OH; Real Food Blends, Chesterton, IN) were tested. Results: Data from individual measurements were aggregated for ENFit and legacy tubes and revealed higher syringe compression force in legacy tubes compared with ENFit tubes for 20Fr size with Jevity formula. Our institution's BTF formula revealed that legacy tubes had lower syringe compression force than ENFit tubes for 14Fr tube size. Remaining measurements revealed no significant difference. Model 1 of regression analysis revealed that only formula and tube size were significant with R2 of 0.63. Model 2 evaluating the impact of tube size, blender, time of blending, and legacy vs ENFit revealed that tube size, blender used, and blending time were significant with legacy vs ENFit being nonsignificant (R2 of 0.72). Conclusions: Overall, only a small number of tube sizes (14Fr and 20Fr) with selected formulas revealed a significant difference between ENFit and Legacy tubes, with remaining studies finding no significant difference. Regression analysis revealed that variables such as formula, size of tube, blender used, and time of blending may have more impact on compression force.