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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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Since telemedicine traditionally has not been integrated into medical training, academic centres have been left to independently develop "just in time" curricula or implement telemedicine services without formal training. This often entails faculty-precepted visits with scheduled patients; however, if participation in telemedicine visits is not available to the learner, simulation or standardised patients can be used to replicate the telemedicine experience. TELEMEDICINE CURRICULUM DEVELOPMENT Now that you have begun to implement telemedicine in your practice, you wonder how best to train your faculty and trainees on appropriate and effective telemedicine usage. Structured observations should include self-reflection from the trainee and formative feedback that addresses telemedicine-specific competencies and corresponding trainee documentation.14 In addition to or in place of OCEs, telemedicine evaluation may take place via OSCE (12, 13). [Extracted from the article]

KIR3DL3-HHLA2 interactions protect tumors: HHLA2 (a B7 family member) has both immune inhibitory and activating abilities and is expressed in many human cancers. These differing functions rely on the receptor to which it binds, yet many of these receptors are uncharacterized. Here, Wei et al. found that KIR3DL3 bound to HHLA2 and was expressed on effector memory CD8+ T cells and CD56dim NK cells. Interactions of KIR3DL3 with tumoral HHLA2 inhibited the function and killing capacity of both CD8+ T cells and NK cells. KIR3DL3+ immune cells infiltrated various types of HHLA2+ tumors from patients with cancer, and blockade of KIR3DL3 inhibited the growth of tumors in various mouse models. Thus, KIR3DL3-HHLA2 inhibits immune-mediated clearance of tumor cells and presents a possible immunotherapeutic target for cancer. 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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

LAG-3, through interaction with a variety of ligands, regulates T cell function via inhibition of T cell proliferation and activation. It has been demonstrated to be overexpressed on tumor infiltrating lymphocytes (TILs) of a variety of cancers with associated poor outcomes. The purpose of this study is to characterize the expression pattern and clinical significance of LAG-3 in pediatric Hodgkin lymphoma (HL). Patient tumor samples from Children's Oncology Group clinical trial AHOD0031 with matched patient outcome data were analyzed for the expression of LAG-3 and PD-L1 using immunohistochemistry. 73/115 patients (63%) demonstrated positive LAG-3 staining. No demographic or survival outcome data were significantly associated with LAG-3 expression. Interestingly, patients with the lowest density of expression were found to have the worst EFS, and those with highest density of expression demonstrated the best EFS. There was a positive statistically significant relationship between presence of LAG-3 and PD-L1 expression. This project is innovative in its characterization of LAG-3 as an immune checkpoint target in pediatric HL. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]