Management of undifferentiated embryonal sarcoma of the liver: A Pediatric Surgical Oncology Research Collaborative study.

BACKGROUND: Undifferentiated embryonal sarcoma of the liver (UESL) is a rare tumor for which there are few evidence-based guidelines. The aim of this study was to define current management strategies and outcomes for these patients using a multi-institutional dataset curated by the Pediatric Surgical Oncology Research Collaborative. METHODS: Data were collected retrospectively for patients with UESL treated across 17 children's hospitals in North America from 1989 to 2019. Factors analyzed included patient and tumor characteristics, PRETEXT group, operative details, and neoadjuvant/adjuvant regimens. Event-free and overall survival (EFS, OS) were the primary and secondary outcomes, respectively. RESULTS: Seventy-eight patients were identified with a median age of 9.9 years [interquartile range [IQR): 7-12]. Twenty-seven patients underwent resection at diagnosis, and 47 patients underwent delayed resection, including eight liver transplants. Neoadjuvant chemotherapy led to a median change in maximum tumor diameter of 1.6 cm [IQR: 0.0-4.4] and greater than 90% tumor necrosis in 79% of the patients undergoing delayed resection. R0 resections were accomplished in 63 patients (81%). Univariate analysis found that metastatic disease impacted OS, and completeness of resection impacted both EFS and OS, while multivariate analysis revealed that R0 resection was associated with decreased expected hazards of experiencing an event [hazard ratio (HR): 0.14, 95% confidence interval (CI): 0.04-0.6]. At a median follow-up of 4 years [IQR: 2-8], the EFS was 70.0% [95% CI: 60%-82%] and OS was 83% [95% CI: 75%-93%]. CONCLUSION: Complete resection is associated with improved survival for patients with UESL. Neoadjuvant chemotherapy causes minimal radiographic response, but significant tumor necrosis.

Survival outcomes in pediatric patients with metastatic Ewing sarcoma who achieve a rapid complete response of pulmonary metastases.

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.

A Social Media Blueprint - Understanding What Makes the Optimal Social Media Account for Paediatric Surgical Families.

BACKGROUND: Social media is one of the most common sources of information for parents seeking information on their child's health. The purpose of this study was to better understand the social media habits of parents of paediatric surgery patients through surveys and focus groups. METHODS: An online survey was distributed to parents visiting paediatric surgery clinics at a tertiary care hospital. Surveys were distributed via QR code and social media. Two virtual focus groups were conducted with parents of paediatric patients. Descriptive statistics were used for survey analysis and focus group transcripts were thematically analyzed. RESULTS: A total of 107 respondents completed the online survey. Median age of respondents was 36 (interquartile range: 32-41). 81.3% of the respondents were female. Facebook was the preferred social media platform (47.2%), followed by Instagram (41.5%) then other platforms (4.7%). Respondents indicated that their preferred length of video was 30 s (41.2%). When asked which type of video content they prefer, participants indicated animated video as most popular (66.0%) followed by a physician speaking (60.0%), and slides with voiceover (45.0%). The focus groups revealed themes of: (1) functionality and content; (2) branding, aesthetic and legitimacy; (3) unmasking of physicians; (4) peer and community support. CONCLUSION: Creating a successful social media account for parents must take into consideration their social media habits. A Facebook account that features brief videos may be most likely to engage parents. Additionally, physicians need to establish credibility and legitimacy of their content to attract their target audience. LEVEL OF EVIDENCE: III.

The Intersection of Family Planning and Perception of Career Advancement in General Surgery.

INTRODUCTION: Women in surgery face unique challenges, particularly as it relates to family planning, parental leave, infant feeding, and career advancement. This study highlights disparities in present day general surgery training to tackle longstanding gender inequities. METHODS: An open, anonymous online survey was distributed to Canadian residents, fellows, and practicing general surgeons through the Canadian Association of General Surgeons e-mail list from November 2021-March 2022. Data were analyzed descriptively and chi-square tests were performed to examine categorical outcomes across gender. RESULTS: A total of 89 general surgery respondents (13.8% response rate) completed the survey (22 cisgender men; 65 cisgender women). Twenty six percent of participants had accessed fertility services or used assistive reproductive technologies. Of the participants with children, 36.4% of men and 100.0% of women took at least one parental leave during residency or clinical practice. A greater proportion of women compared to men agreed that their training/practice influenced their decision to have children (P = 0.002) and when to have children (P < 0.001). Similarly, a greater proportion of women indicated they had concerns about future family planning (P = 0.008), future fertility (P = 0.002), and future parental leave (P = 0.026). Fifty nine percent of women and zero men agreed that taking parental leave impacted their career advancement (P = 0.04). CONCLUSIONS: Women surgeons and surgical trainees continue to face challenges with respect to family planning, parental leave, infant feeding, and career advancement. Further research is needed to explore the experiences of women surgeons. By providing surgeons with the support required to achieve their family planning goals, surgeons can accomplish their family and career goals with less conflict.

Management and Outcomes of Wilms Tumor With Suprarenal Intravascular Extension: A Pediatric Surgical Oncology Research Collaborative Study.

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.

Comparison of Outcomes Between Surveillance Ultrasound and Completion Lymph Node Dissection in Children and Adolescents With Sentinel Lymph Node-Positive Cutaneous Melanoma.

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.

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.

Across the Twitter-verse: Is Twitter an equitable tool in academic medicine? A scoping review.

The goal of this study was to investigate what is known about the demographic characteristics of Twitter influencers in academic medicine. We conducted a literature search and scoping review exploring the demographic characteristics of Twitter influencers in academic medicine. Included studies evaluated Twitter influence by any metric and reported associated demographic characteristics. There were no date or language restrictions. Data points included metrics of influence, demographics, and study characteristics. From 1656 records, thirteen met inclusion criteria. Influence was variably defined based on followers, measures of centrality, measures of engagement, or through third-party software. Studies were conducted at single time points and relied heavily on facial recognition for classification of sex or gender, with no studies exploring race as a variable of interest. Twitter influencers in academic medicine were found to be disproportionately male: significantly fewer influencers were female at all levels of professional status. Two studies identified more female influencers among trainee populations. Female users were less likely to develop influence based on followers, retweets, likes, or other measures of engagement at academic meetings, despite equal or greater representation at these meetings. Results on associations between Twitter influence and number of publications or h-index varied considerably. No included studies explored race or ethnicity in relation to Twitter influence, which presents a significant gap in the literature. Our findings highlight the need for larger, user-engaged studies of inequities related to social media influence in academic medicine, especially as social media is increasingly incorporated into academic portfolios.

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.

A comparison of balloon and nonballoon gastrostomy tubes in children.

PURPOSE: The purpose of this study was to compare primary outcomes following insertion of balloon and nonballoon gastrostomy tubes (G-tubes). METHODS: A retrospective chart review over a 5-year period comparing the need for emergency, radiologic, or operative interventions between balloon and nonballoon G-tube devices was performed. RESULTS: 145 patient charts were reviewed (46.8% female, 53.1% male). The indication for G-tube insertion was failure to thrive in 83.4%. Average age at insertion was 4.3 years (0-17.9 years). 37.2% had a balloon type G-tube, and 62.8% had a nonballoon type. Patients with a nonballoon device had 1.14 (0-15) ER visits related to the G-tube vs. 0.48 (0-6) visits with a balloon device. Of the ER visits for patients with a nonballoon device, 26.9% were replaced in ER, 38.5% in radiology, and 34.6% required an operation for replacement. For patients with a balloon device, 47.8% were replaced in the ER, 52.2% were replaced in radiology (GJ), and none required operative replacement. The majority of patients who initially had a nonballoon G-tube placed required a second operation for device change (95.7%). Patients with nonballoon devices required significantly more operations (average 2.55, range 0-16) vs patients with balloon devices (average 0.40, range 0-3) (p < .05). CONCLUSIONS: Balloon-type G-tubes require less ER visits and operative interventions compared to nonballoon G-tubes. LEVEL OF EVIDENCE: C.

Compassion Fatigue in Surgical Trainees.

OBJECTIVE: Compassion fatigue (CF) is the profound sense of emotional exhaustion that care providers can experience as the result of helping others in distress. CF can contribute to burnout (BO), depression, and stress-related illness. While surgeons and surgical trainees may be at high risk for developing CF, it has not been adequately characterized or explored in this population. The objective of this study was to examine the prevalence and impact of CF in surgical trainees with a view to inform a management strategy. STUDY DESIGN AND SETTING: A mixed method study was conducted using survey and interview methods. An email survey including the Professional Quality of Life Scale Version 5, an instrument to assess CF, was sent to all trainees in the Department of Surgery at the University of Toronto. Survey data were analyzed descriptively and using one-sample t tests. Semistructured interviews were conducted with volunteered trainees. Data collection and analysis occurred iteratively and inductively using the constant comparison method. RESULTS: One hundred fifteen trainees completed the survey representing a 47% response rate. Ninety-nine respondents (40.7%) completed the Professional Quality of Life Scale tool. The mean score on the compassion satisfaction subscale was 36.9 (SD 6.7), on the BO subscale was 26.2 (SD 5.6), and on the secondary traumatic stress (STS) subscale was 21.2 (SD 6.3). The mean on the compassion satisfaction subscale was not statistically different from the population mean (p = 0.22). The means for the BO and STS scales were statistically higher in our study sample compared to the normative data (p < 0.0001 for each). Thematic qualitative findings indicated trainees experienced CF symptoms. Participants described systemic barriers to mitigating CF including workload and a cultural expectation to be unemotional at work. CONCLUSION: Surgical trainees report high levels of BO and STS and currently use informal coping strategies outside of their academic and hospital environments. Trainees are likely to welcome and benefit from an organized response to support their emotional health when facing difficult patient encounters.

Long-term outcomes for children with very early-onset colitis: Implications for surgical management.

PURPOSE: The timing of J-pouch surgery following colectomy for children with very early-onset colitis is controversial, with some advocating early reconstruction and others delaying reconstruction because of fear that the colitis may be owing to Crohn's disease (CD). We sought to determine the long-term incidence of CD in this population and whether there may be clinical features that predict the risk of CD. METHODS: Children with noninfectious colitis diagnosed prior to age 10, who underwent subtotal colectomy and ileostomy from 2000 to 2015, were reviewed. RESULTS: Twenty-five children were identified. Median age at presentation was 5.4years. Four were initially diagnosed with CD (16%), 14 with ulcerative colitis (UC) (56%), and 7 with inflammatory bowel disease unclassified (IBD-U) (28%). Eight eventually had pouch surgery. Five of the children with an initial diagnosis of UC or IBD-U developed findings that changed the diagnosis to CD at a median age of 13.4 (range 10.3 to 16.7) years. None had any indicators of CD at the initial presentation. CONCLUSIONS: Approximately one quarter of patients with very early-onset colitis originally diagnosed as UC or IBD-U had a reclassification in diagnosis to CD over time. J-pouch reconstruction should be delayed until adolescence in children with very early-onset colitis. LEVEL OF EVIDENCE: 2C.

The role of imaging in the preoperative assessment of children with inflammatory colitis.

BACKGROUND: Accurate diagnosis of Crohn's disease or ulcerative colitis is essential for surgical planning. This study compared the accuracy, safety, and utility of small bowel follow-through (SBFT) and magnetic resonance enterography (MRE) for detecting small bowel (SB) involvement preoperatively. METHODS: A retrospective review of children who underwent colectomy for inflammatory bowel disease (2000-2014) was performed. Preoperative SBFT and MRE were independently reviewed by two radiologists blinded to clinical data. Gold standard for diagnosis was surgical pathology. RESULTS: 68 patients (36 female) were included. 45 patients had SBFT prior to colectomy, 17 patients had MRE, and 6 both. Interrater reliability for radiologic interpretation was 90% (SBFT) and 91% (MRE). Mean study durations were 190min (SBFT) and 59min (MRE). Median effective dose for SBFT was 1.5mSv, while MRE involved no ionizing radiation. Specificities for diagnosing SB involvement were 87.5% (SBFT) and 94% (MRE). 54 patients underwent subtotal colectomy and ileostomy, 12 restorative proctocolectomy, and 2 colectomy with ileorectal anastomosis. Preoperative imaging correlated with surgical pathology in 83% (SBFT) and 90% (MRE). CONCLUSION: MRE is at least as effective as SBFT for assessing SB disease in children with colitis prior to colectomy. MRE requires less time and does not expose children to ionizing radiation. Retrospective Case Series, Level 4 Evidence.

The operative management of children with complex perianal Crohn's disease.

BACKGROUND/PURPOSE: Perianal Crohn's disease (PCD) can affect both quality of life and psychological wellbeing. A subset of pediatric patients with complex PCD require surgical intervention, although appropriate timing and treatment regimens remain unclear. This study aimed to describe a large pediatric cohort in a tertiary center to determine the range of surgical management in children with complex PCD. METHODS: A retrospective review of children requiring operative intervention for PCD over 13 years (2002-2014) was performed. PCD was divided into simple and complex based on the type of surgical procedure, and the two groups were compared. RESULTS: The 57 children were divided into two groups: the simple group (N=43) underwent abscess drainage ± seton insertion alone, and the complex group (N=14) underwent loop ileostomy ± more extensive surgery. In the complex group, females were more predominant (57% of complex vs 30% of simple), and the average age at diagnosis was lower. Anti-TNF therapy was utilized in 79.1% of simple and 100% of complex PCD. All 14 complex patients underwent a defunctioning ileostomy, with 7 requiring further operations (subtotal colectomy=4, proctocolectomy ± anal sparing=5, plastic surgery reconstruction with perineal flap/graft=4). CONCLUSION: Complex PCD represents a small but challenging subset of patients in which major surgical intervention may be necessary to alleviate the symptoms of this debilitating condition. LEVEL OF EVIDENCE: retrospective case study with no control group - level IV.

Women in academic surgery: why is the playing field still not level?

BACKGROUND: The purpose of this study was to explore career satisfaction and advancement for women in academic surgery. METHODS: A 48-item web-based survey was emailed to women surgeons in academic centers across Canada, exploring career advancement, family planning, mentorship, discrimination, and career satisfaction. RESULTS: The survey response rate was 38% (81 of 212); 18% of participants felt they experienced gender discrimination in medical school, 36% in residency, 12% in fellowship, and 41% as staff surgeons. More than half felt that their gender had played a role in the career challenges they faced. Responses to open-ended questions suggested that many surgeons struggled to balance their academic careers with family life. Despite this, participants rated their career satisfaction very highly. CONCLUSIONS: There remain ongoing challenges for women in academic surgery including lack of gender equality, appropriate mentorship, and accommodations for surgeons with families. Continued advancement of women in academic surgery is dependent on addressing these concerns.

The role of surgery for children with perianal Crohn's disease.

PURPOSE: Children with perianal Crohn's disease (PCD) are a unique and diverse patient population. The purpose of this study was to describe the spectrum of disease and role of surgery. METHODS: A retrospective chart review of all children having at least one surgical intervention for PCD over 10 years was performed. RESULTS: Fifty-seven patients (63% male) aged 0.5-17 (median 13) years were identified. Perianal disease consisted of skin tags (49%), superficial fistulae (49%), deep fistulae (37%), superficial abscesses (68%), deep abscesses (9%), skin breakdown (19%), and anal strictures (7%). 84% received anti-TNF therapy, with 27% treated with a second anti-TNF medication. Minor surgical procedures, commonly done during anti-TNF therapy, included abscess drainage (67%) and seton placement (33%). Major surgical procedures, done almost exclusively after anti-TNF failure, included defunctioning ileostomy (23%) and subtotal colectomy (9%). Follow-up ranged from 7 to 160 (median 54) months. CONCLUSIONS: Pediatric PCD has a wide range of disease severity. Minor surgery provides adequate drainage before and during anti-TNF therapy, while major surgery plays a role in medically refractory disease. Appropriate surgical intervention remains an important part of the treatment paradigm.

Effects of high pressure on azobenzene and hydrazobenzene probed by Raman spectroscopy.

In this study, two hydrazine derivatives, azobenzene and hydrazobenzene, were compressed in a diamond anvil cell at room temperature up to 28 GPa followed by decompression. In situ Raman spectroscopy was employed to monitor the pressure-induced structural evolutions. Azobenzene was found to undergo a phase transition at ~10 GPa. Further compression to 18 GPa resulted in an irreversible breakdown of the molecular structure. Although hydrazobenzene exhibited a structural transition at a similar pressure of 10 GPa, it was found to sustain a compression pressure as high as 28 GPa without chemical reactions. The transition sequence of hydrazobenzene upon compression and decompression was thus entirely reversible in the pressure region studied, in strong contrast to that of azobenzene. The high-pressure structures of these two molecules were examined based on the spectroscopic data, and their drastically different high-pressure behaviors were analyzed and interpreted with the aid of ab initio molecular orbital calculations.