Epithelial Predominant Wilms Tumor in an Adult Patient: Case Report and Literature Review.

Although rare in adults, Wilms tumor is the most common pediatric renal tumor. Treatment typically involves radical nephrectomy followed by adjuvant chemotherapy or radiation, although outcomes differ between children and adults which may be due to challenges in accurately diagnosing these patients. In this article, we present a case report of an adult patient with Jeune syndrome and multiple urologic abnormalities who underwent radical nephrectomy for a large renal mass and was subsequently diagnosed with an epithelial predominant Wilms tumor. Epithelial predominant Wilms tumor may have distinct origins from other Wilms tumor histological subtypes and may incur better outcomes. Herein, we discuss the literature surrounding this rare entity as well as the anticipated treatment course.

Preventing Infection in Pediatric Patients Receiving Chemotherapy: A Survey of Provider Recommendations.

PURPOSE: Sepsis is the leading cause of mortality in patients with childhood cancer receiving cytotoxic chemotherapy. Pediatric hematology/oncology and transplant (PHOT) providers must counsel their patients on the safety of public activities and weigh the risk of infection exposure with the social and developmental benefits of in-person school and social outings. We hypothesize that there is significant variability in recommendations given by PHOT providers. METHODS: An electronic anonymous survey was developed and piloted by a group of PHOT providers to assess current methods for educating patients and families on limiting infectious exposures. Five clinical vignettes were created by the study team to explore how providers balance the competing priorities of safety and health-related quality of life (HRQoL). The electronic survey was institutional review board-approved and disseminated via email to all PHOT providers affiliated with the Children's Oncology Group across the United States. RESULTS: In total, 545 clinicians completed the survey. Most respondents were attending physicians (393, 72%), followed by fellows (61, 11%), advanced practice providers (APPs; 38, 7%), and nurses (37, 7%). On average, nurses and fellows made more conservative recommendations for avoiding infectious exposures compared with the recommendations from attending physicians and APPs (P < .0001). On average, providers with more years of clinical experience expressed less cautious recommendations, whereas those with less years of experience provided more cautious recommendations for avoiding infectious exposures (P = .0072). CONCLUSION: This survey demonstrates the importance of collaboration between all members of the care team in defining priorities for balancing safety risk and HRQoL to provide consistent messaging to patients. The variations in survey responses highlight the need for universal guidelines to standardize physician recommendations for limiting infectious exposures in pediatric patients on chemotherapy.

Child Health and the Pediatric Hematology-Oncology Workforce: 2020-2040.

Pediatric hematology-oncology (PHO) is 1 of the oldest recognized pediatric subspecialities. PHO physicians care for infants, children, adolescents, and young adults with all types of cancer and nonmalignant blood conditions, in many cases temporarily assuming the role of a primary care physician because of the complexity and intensity of treatment. However, the number of clinically active PHO subspecialists needed to care for children in the United States remains unknown. Recent papers suggest a potential oversaturation of PHO physicians in some geographic areas. This article is part of a Pediatrics supplement focused on projecting the future supply of the pediatric subspecialty workforce. It draws on information available in the literature, data from the American Board of Pediatrics, and findings from a new microsimulation model estimating the future supply of pediatric subspecialists through 2040. The model predicts a workforce growth in PHO subspecialists of 66% by 2040. Alternative scenarios, including changes in clinical time and fellowship size, resulted in a difference in growth of ±18% from baseline. The model also forecasts significant geographic maldistribution. For example, the current workforce is concentrated in the Northeast Census region and the model predicts the New England Census division will have a 2.9-fold higher clinical workforce equivalent per 100 000 children aged 0 to 18 years than the Mountain Census division by 2040. These findings suggest potential opportunities to improve the PHO subspecialty workforce and the outcomes and experiences of its patient population through educational changes, practice initiatives, policy interventions, and dedicated research.

Gaps in Adolescent and Young Adult Cancer Education in Oncology Fellowship Training.

Purpose: Adolescents and young adults (AYAs, 15-39 years) with cancer experience disparities in care and outcomes compared with older/younger patients. AYAs receive care from medical and pediatric oncologists, however, little is known about the extent of training fellows receive. This needs assessment evaluating current AYA oncology (AYA-O) education in pediatric and medical oncology fellowship programs to identify knowledge gaps for curricular development. Methods: An anonymous, cross-sectional, web-based survey developed by pediatric and medical oncologists was sent to medical (n = 178) and pediatric (n = 119) hematology/oncology program directors (PDs) at 251 sites in the United States. PDs were asked to participate and distribute the survey to their fellows. Survey questions addressed current AYA curriculum, provider comfort, and priorities for future AYA educational content. Results: Participants from 69/251 programs responded (program response rate = 27%), including 51 PDs (32 pediatric, 19 medical oncology) and 58 fellows (33 pediatric, 25 medical oncology). Eighty-five percent of PDs (44/51) reported lacking formal AYA curricula. Of these, 80% (35/44) offer some topic-specific lectures, while 20% (9/44) provide little/no education for any topics. For nearly all topics, at least 45% of combined respondents reported little/no education. Respondents believe AYA topics are important for inclusion in future curricula. The most important topics for inclusion reported were oncofertility (82%), survivorship (78%), and communication (77%). Conclusions: There are large and actionable gaps in AYA-O education during fellowship training. Efforts are underway to develop AYA-O curriculum to provide both medical and pediatric oncology fellows with the knowledge and skills required to provide optimal AYA care.

"What's My Line?": Pseudo-Improvised Teaching When the Clinical Teaching Script Is Blank.

ABSTRACT: Commitment to clinical education often requires significant forethought and attention to provide a comprehensive learning experience for trainees. In these settings, teaching is typically time-limited, prompted by a clinical scenario, and requires preparation. However, it is not uncommon for teachers to have insufficient time to prepare or to encounter a clinical scenario in which they have not yet developed a teaching script.In this article, the authors share 5 categories of teaching techniques that instructors can pull from regardless of the prompt or busyness of the clinical setting and that are ideal for using when the teaching script is "blank." They call this approach of having scenario-independent teaching techniques ready to be applied with minimal preparation, "pseudo-improvised teaching."Drawing from the literature, their own experience, and borrowing from improvisational theater, the authors share a toolkit of pseudo-improvised teaching techniques spanning from pathophysiology to clinical skills to work-life integration. In addition to highlighting several techniques, they describe models of meta-structure for teaching in which the use of themes for the day (i.e., longitudinal themes) and routines can ease some of the cognitive load felt by both learners and educators.

Perceptions of the stressful job search for pediatric hematology/oncology fellows.

The pediatric hematology and oncology (PHO) workforce landscape has been evolving over the past decades, with concern for waning interest in the subspecialty. We aimed to evaluate the impact of the initial PHO job search on fellow stress and anxiety, in addition to perceptions of the job search experience and potential areas of improvement. An anonymous survey consisting of demographics, emotional health impacts such as stress and anxiety, and job search experiences was developed and distributed to program leadership at all 74 PHO fellowship programs. At least one representative from 49 (66%) programs responded. Faculty were less likely than fellows to perceive that fellows are struggling to find jobs (p = .0198). However, faculty were more likely than fellows to perceive that fellows are either "extremely stressed" or "stressed" due to job search (p = .0003). By June 2021, 30 of 44 (68%) candidates had been offered a position, and 80% of those had accepted. There were no significant associations between fellow stress level and job offering, proximity to their ideal goals, or change in career type. Common barriers to the job search included geographic constraints and partner employment. Respondents identified centralized job listings, formal training on career development strategies, introduction to various career paths, and more transparency about the search as needed interventions. The perception of difficulty and stress regarding the job search is endorsed by most, identifying a need for more interventions to improve the experience. These data also highlight unmet mentorship and educational needs among PHO fellows in preparing for the job search.

v-SYMPHONY career development series: A collaboration to enhance professional awareness for pediatric hematology oncology trainees.

BACKGROUND: A recent survey of pediatric hematology oncology (PHO) physicians identified that a majority believe fellows are struggling to find jobs that align with their goals. Career development for trainees has historically been home institution-specific, limiting fellows' exposures to career path possibilities. The "virtual-Symposium of Pediatric Hematology/Oncology of New York (v-SYMPHONY)" instituted a tristate Career Development Series for PHO trainees to better address their needs and increase awareness of the variety of PHO career opportunities. PROCEDURE: The v-SYMPHONY Career Development Series incorporated three sessions: (a) institutional perspective, (b) individual perspectives, and (c) nuts and bolts of job search. Pre- and post-series surveys were administered to participants to measure impact. RESULTS: Forty-one fellows registered for the series and completed a pre-survey. Over half (54%) were in their third or later year of fellowship. Careers with a clinical focus were the most commonly desired career path (59%). Most had received career development advice only from faculty within their institutions (90%). Post-surveys were completed by 11 PHO fellows. Overall, 100% of respondents reported benefiting from the career sessions and recommended the series should be repeated annually. Over 90% learned new information to prepare for the job search. CONCLUSIONS: The v-SYMPHONY Career Development Series for PHO fellows across multiple institutions was established and was extremely well received by its participants. PHO fellows agreed that these sessions were beneficial in helping prepare them for the job search process. An annual regional Career Development Series is feasible and is strongly suggested to support PHO fellows.

Chemotherapy Refractory Spindle-Cell Rhabdomyosarcoma of the Bladder Treated With Consolidative Ovarian-Sparing Radical Surgery.

We present a case of chemotherapy refractory spindle cell rhabdomyosarcoma of the lower urinary tract in a 15-month-old female that ultimately required consolidative surgery with cystectomy, urethrectomy, ovarian-sparing hysterectomy, bilateral salpingectomy, anterior vaginal wall resection, and bilateral pelvic lymph node dissection. Genitourinary reconstruction was performed by ileal conduit creation and vaginoplasty. After completion of her maintenance postoperative chemotherapy regimen, the patient has remained disease-free for approximately 27 months.

Can you hear me now? Tools for cultivating a culture of respect, value, and appreciation within pediatric hematology, oncology, and cellular therapy.

The American Society of Pediatric Hematology/Oncology (ASPHO) conducted a workshop "Can you hear me now? Cultivating a culture of respect, value, and appreciation within pediatric hematology/oncology" at their annual meeting in May 2022 in hopes of exploring how the members can enhance wellness in a climate of increasing diversity. Initiatives in the past have focused on personal care, but it has been widely shown that administrative and institutional driven initiatives are essential to create an environment of wellness. In this interactive workshop, we discovered that 22% of participants felt their institution does not instill a culture of respect. We offered tools to the audience on multiple levels: graceful self-promotion, diversity and inclusion, and leadership perspective on creating a culture of respect to address the individual, local community, and top-down leadership approaches. Here, we offer a summary on the content of the workshop, and expand upon many of the discussion points that were raised during the workshop. We bring forth novel information on each topic individually from diverse points of view, specific to the field of pediatric hematology/oncology (PHO). We aim to highlight the importance of creating a diverse and respectful work environment in PHO in hopes of ensuring motivated, satisfied, and fulfilled healthcare providers who feel appreciated and valued.

Social Media and Professional Development for Oncology Professionals.

The use of social media continues to increase in health care and academia. Health care practice, particularly the oncologic field, is constantly changing because of new knowledge, evidence-based research, clinical trials, and government policies. Therefore, oncology trainees and professionals continue to strive to stay up-to-date with practice guidelines, research, and skills. Although social media as an educational and professional development tool is no longer completely new to medicine and has been embraced, it is still under-researched in terms of various outcomes. Social media plays several key roles in professional development and academic advancement. We reviewed the literature to evaluate how social media can be used for professional development and academic promotion of oncology professionals.

Provider and staff crisis well-being associated with trust in leadership and baseline burnout.

BACKGROUND: The impact of the coronavirus 2019 (COVID-19) pandemic on the emotional health of health care workers continues to be an area of active research. However, few studies have focused on those working in pediatrics and its subspecialties, as well as ancillary and non-patient-facing staff. The purpose of this study was to determine the prevalence and associated predictors of burnout and emotional well-being of providers and staff. METHODS: An anonymous electronic survey was developed evaluating demographics, pandemic experiences, possible predictor variables, and three main outcomes of burnout, psychological distress, and perceived stress. Pediatric hematology oncology (PHO) chiefs and program directors across the country were invited to participate and disseminate the survey to their programs. RESULTS: A total of 682/1950 (35% of invited) individuals responded to all predictor and outcome variables. Over half reported high levels of burnout and some reported moderate/high levels of distress. Prepandemic burnout and decreased trust in leadership were associated with all three outcomes. Additional predictors included having a child ≤18 years at home, hospital role, and worrying about patient care or relationship with their patients. The majority (n = 444/682, 65.5%) reported that their institution had made COVID-19-related mental health resources available. However, only 6.5% (n = 44/682) reported utilizing these resources. CONCLUSIONS: While the majority of PHO providers and staff were resilient during the early stages of the COVID-19 pandemic, many reported high levels of burnout, yet few are utilizing institutional resources. This study has highlighted several actionable areas to help identify and address factors that are wearing down the emotional well-being of providers and staff.

Perception of Medical Student Mistreatment: Does Specialty Matter?

PURPOSE: Medical student mistreatment is pervasive, yet whether all physicians have a shared understanding of the problem is unclear. The authors presented professionally designed trigger videos to physicians from 6 different specialties to determine if they perceive mistreatment and its severity similarly. METHOD: From October 2016 to August 2018, resident and attending physicians from 10 U.S. medical schools viewed 5 trigger videos showing behaviors that could be perceived as mistreatment. They completed a survey exploring their perceptions. The authors compared perceptions of mistreatment across specialties and, for each scenario, evaluated the relationship between specialty and perception of mistreatment. RESULTS: Six-hundred fifty resident and attending physicians participated. There were statistically significant differences in perception of mistreatment across specialties for 3 of the 5 scenarios: aggressive questioning (range, 74.1%-91.2%), negative feedback (range, 25.4%-63.7%), and assignment of inappropriate tasks (range, 5.5%-25.5%) (P ≤ .001, for all). After adjusting for gender, race, professional role, and prior mistreatment, physicians in surgery viewed 3 scenarios (aggressive questioning, negative feedback, and inappropriate tasks) as less likely to represent mistreatment compared with internal medicine physicians. Physicians from obstetrics-gynecology and "other" specialties perceived less mistreatment in 2 scenarios (aggressive questioning and negative feedback), while family physicians perceived more mistreatment in 1 scenario (negative feedback) compared with internal medicine physicians. The mean severity of perceived mistreatment on a 1 to 7 scale (7 most serious) also varied statistically significantly across the specialties for 3 scenarios: aggressive questioning (range, 4.4-5.4; P < .001), ethnic insensitivity (range, 5.1-6.1; P = .001), and sexual harassment (range, 5.5-6.3; P = .004). CONCLUSIONS: Specialty was associated with differences in the perception of mistreatment and rating of its severity. Further investigation is needed to understand why these perceptions of mistreatment vary among specialties and how to address these differences.

Supporting Health-Care Workers and Patients in Quarantine Wards: Evidence From a Survey of Frontline Health-Care Workers and Inpatients With COVID-19 in Wuhan, China.

Objective: Frontline health-care workers and patients with COVID-19 have been identified as high-risk groups for psychological problems. Experience of working or staying in quarantine wards generated psychological stressors for health-care workers and patients with COVID-19. The present study aimed to investigate the psychological symptoms of hospitalized patients with COVID-19 and the health-care workers treating them during the outbreak period, examine the effects of psychological stressors on mental health in both populations and perceived coping resources for both sides. Methods: Three hundred and eleven health-care workers working in a COVID-19 designated hospital in Wuhan, China, and 148 hospitalized patients with COVID-19 in the same hospital participated in this cross-sectional survey conducted in February 2020. Psychological symptoms, psychological stressors, and perceived coping resources were reported by both groups. Results: Thirty-three percent of health-care workers and 35.2% of patients with COVID-19 had significant psychological symptoms that were indicative of a high risk for psychological disorders. Pandemic-related psychological stressors contributed to psychological symptoms for both populations. Concern about patients was one aspect of psychological stressors of frontline health-care workers and both groups perceived support from the opposite side as an important external coping resource. Conclusion: The results shed light on the need to provide psychological support to both frontline health-care workers and patients with COVID-19 and suggest enhancing the treatment alliance might be effective to improve mental health for both populations during the crisis.

KIR3DL3-HHLA2 is a human immunosuppressive pathway and a therapeutic target.

The B7 family ligand HERV-H LTR-associating protein 2 (HHLA2) is an attractive target for cancer immunotherapy because of its coinhibitory function, overexpression in human cancers, and association with poor prognoses. However, the knowledge of the HHLA2 pathway is incomplete. HHLA2 has an established positive receptor transmembrane and immunoglobulin (Ig) domain containing 2 (TMIGD2) but a poorly characterized negative receptor human killer cell Ig-like receptor, three Ig domains, and long cytoplasmic tail (KIR3DL3). Here, KIR3DL3 and TMIGD2 simultaneously bound to different sites of HHLA2. KIR3DL3 was mainly expressed on CD56dim NK and terminally differentiated effector memory CD8+ T (CD8+ TEMRA) cells. KIR3DL3+ CD8+ TEMRA acquired an NK-like phenotype and function. HHLA2 engagement recruited KIR3DL3 to the immunological synapse and coinhibited CD8+ T and NK cell function and killing, inducing immune-evasive HHLA2+ tumors. KIR3DL3 recruited SHP-1 and SHP-2 to attenuate Vav1, ERK1/2, AKT, and NF-κB signaling. HHLA2+ tumors from human kidney, lung, gallbladder, and stomach were infiltrated by KIR3DL3+ immune cells. KIR3DL3 blockade inhibited tumor growth in multiple humanized mouse models. Thus, our findings elucidated the molecular and cellular basis for the inhibitory function of KIR3DL3, demonstrating that the KIR3DL3-HHLA2 pathway is a potential immunotherapeutic target for cancer.

COVID-19 has changed the way we think about training future pediatric hematologists/oncologists.

COVID-19 has upended medical practice and education, but has also catalyzed enhancements in the field. Early on, a local group of researchers united to investigate the impact of the pandemic on pediatric hematology oncology (PHO). From this group, a regional educational series was established, "virtual-Symposium of Pediatric Hematology/Oncology of New York" (v-SYMPHONY). The implementation of these endeavors while PHO fellowship applications are declining has highlighted our perceptions that education, mentoring, and career expectations are not keeping up with the needs of current trainees. We describe our regional experience joining together to further education and research, and reflect on the current landscape of PHO training and workforce.

Well-Being of Pediatric Hematology Oncology Providers and Staff During the COVID-19 Pandemic in the New York and New Jersey Epicenter.

PURPOSE: The COVID-19 pandemic has significantly affected the well-being of healthcare workers to varying degrees. The aim of the current study was to investigate how the pandemic has affected the burnout, stress, and emotional well-being of pediatric hematology oncology (PHO) providers and staff in the New York and New Jersey epicenter. METHODS: The study was conducted in June 2020 during the pandemic through an electronic survey. The survey contained questions surrounding demographics, pandemic experiences, and validated burnout, stress, and emotional well-being measures. RESULTS: Two hundred fifty-two PHO providers and staff responded to the survey. Overall, half of the participants reported high levels of burnout, average stress scores were in the mild-moderate range, and the majority scored in the none-to-mild symptomatology range for their well-being. Self-reported burnout levels before the pandemic and geographic work location were statistically significant risk factors for all outcomes. Additional predictors for some outcomes included hospital role, lack of trust in leadership, and deployment. The majority of participants (87.0%) reported that their hospitals had made mental health resources related to COVID-19 available to them but only 8.4% reported having used them. CONCLUSION: PHO providers and staff in the NYC/NJ area are experiencing a range of emotional experiences during the COVID-19 pandemic, but the majority are not using current resources. With the continuation of the pandemic, we must continue efforts to improve provider and staff distress to mitigate the degree of potential negative short-term and long-term impact.