Genetically Modified Porcine-to-Human Cardiac Xenotransplantation.

A 57-year-old man with nonischemic cardiomyopathy who was dependent on venoarterial extracorporeal membrane oxygenation (ECMO) and was not a candidate for standard therapeutics, including a traditional allograft, received a heart from a genetically modified pig source animal that had 10 individual gene edits. Immunosuppression was based on CD40 blockade. The patient was weaned from ECMO, and the xenograft functioned normally without apparent rejection. Sudden diastolic thickening and failure of the xenograft occurred on day 49 after transplantation, and life support was withdrawn on day 60. On autopsy, the xenograft was found to be edematous, having nearly doubled in weight. Histologic examination revealed scattered myocyte necrosis, interstitial edema, and red-cell extravasation, without evidence of microvascular thrombosis - findings that were not consistent with typical rejection. Studies are under way to identify the mechanisms responsible for these changes. (Funded by the University of Maryland Medical Center and School of Medicine.).

Graft dysfunction in compassionate use of genetically engineered pig-to-human cardiac xenotransplantation: a case report.

BACKGROUND: A genetically engineered pig cardiac xenotransplantation was done on Jan 7, 2022, in a non-ambulatory male patient, aged 57 years, with end-stage heart failure, and on veno-arterial extracorporeal membrane oxygenation support, who was ineligible for an allograft. This report details our current understanding of factors important to the xenotransplantation outcome. METHODS: Physiological and biochemical parameters critical for the care of all heart transplant recipients were collected in extensive clinical monitoring in an intensive care unit. To ascertain the cause of xenograft dysfunction, we did extensive immunological and histopathological studies, including electron microscopy and quantification of porcine cytomegalovirus or porcine roseolovirus (PCMV/PRV) in the xenograft, recipient cells, and tissue by DNA PCR and RNA transcription. We performed intravenous immunoglobulin (IVIG) binding to donor cells and single-cell RNA sequencing of peripheral blood mononuclear cells. FINDINGS: After successful xenotransplantation, the graft functioned well on echocardiography and sustained cardiovascular and other organ systems functions until postoperative day 47 when diastolic heart failure occurred. At postoperative day 50, the endomyocardial biopsy revealed damaged capillaries with interstitial oedema, red cell extravasation, rare thrombotic microangiopathy, and complement deposition. Increased anti-pig xenoantibodies, mainly IgG, were detected after IVIG administration for hypogammaglobulinaemia and during the first plasma exchange. Endomyocardial biopsy on postoperative day 56 showed fibrotic changes consistent with progressive myocardial stiffness. Microbial cell-free DNA testing indicated increasing titres of PCMV/PRV cell-free DNA. Post-mortem single-cell RNA sequencing showed overlapping causes. INTERPRETATION: Hyperacute rejection was avoided. We identified potential mediators of the observed endothelial injury. First, widespread endothelial injury indicates antibody-mediated rejection. Second, IVIG bound strongly to donor endothelium, possibly causing immune activation. Finally, reactivation and replication of latent PCMV/PRV in the xenograft possibly initiated a damaging inflammatory response. The findings point to specific measures to improve xenotransplant outcomes in the future. FUNDING: The University of Maryland School of Medicine, and the University of Maryland Medical Center.

Duration of monitoring after cessation of oxygen therapy in infants with bronchiolitis.

AIM: There is no evidence for how long bronchiolitis patients should be observed after coming off oxygen therapy and wide practice variation exists. We aimed to investigate whether it is safe to discharge bronchiolitis patients 4 h after cessation of oxygen therapy. METHODS: A retrospective single-centre cohort study of 884 infants (n = 462 in 2018 vs. n = 422 in 2019) aged 0-24 months admitted with bronchiolitis in 2018 and 2019 was conducted after implementation of a bronchiolitis protocol recommending discharge home 4 h post-cessation of oxygen therapy in 2019. We compared the rate of readmissions and Clinical Reviews/Rapid Responses in the pre- and post-exposure cohorts. RESULTS: There was a significant reduction in median (interquartile range (IQR)) time to discharge post oxygen cessation by 87 min (510 (370-1033) min versus 423 (273-904) min; P < 0.001) and in median (IQR) length of stay by 6.7 h (2.11 (1.54-2.97) days vs. 1.83 (1.17-2.71) days; P < 0.001). There was no significant difference between readmissions in 2018 compared to 2019 (0.6% vs. 1.4%; P = 0.317). In 2018, there were two Clinical Reviews and in 2019 there were two Rapid Responses post-cessation of oxygen. There were 89 patients discharged within 4 h of cessation of oxygen therapy (n = 18 in 2018 vs. n = 71 in 2019; P < 0.001) with no readmissions, Clinical Reviews or Rapid Responses in the 2019 cohort. CONCLUSIONS: This study demonstrates that patients can be discharged 4 h after cessation of supplemental oxygen without increased risk of adverse events.

Ischemia reperfusion injury facilitates lung allograft acceptance through IL-33-mediated activation of donor-derived IL-5 producing group 2 innate lymphoid cells.

Pathways regulating lung alloimmune responses differ from most other solid organs and remain poorly explored. Based on our recent work identifying the unique role of eosinophils in downregulating lung alloimmunity, we sought to define pathways contributing to eosinophil migration and homeostasis. Using a murine lung transplant model, we have uncovered that immunosuppression increases eosinophil infiltration into the allograft in an IL-5-dependent manner. IL-5 production depends on immunosuppression-mediated preservation of donor-derived group 2 innate lymphoid cells (ILC2). We further describe that ischemia reperfusion injury upregulates the expression of IL-33, which functions as the dominant and nonredundant mediator of IL-5 production by graft-resident ILC2. Our work thus identifies unique cellular mechanisms that contribute to lung allograft acceptance. Notably, ischemia reperfusion injury, widely considered to be solely deleterious to allograft survival, can also downregulate alloimmune responses by initiating unique pathways that promote IL-33/IL-5/eosinophil-mediated tolerance.

COVID-19 in New South Wales children during 2021: severity and clinical spectrum.

OBJECTIVES: To describe the severity and clinical spectrum of coronavirus disease 2019 (COVID-19) in children during the 2021 New South Wales outbreak of the Delta variant of the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). DESIGN, SETTING: Prospective cohort study in three metropolitan Sydney local health districts, 1 June - 31 October 2021. PARTICIPANTS: Children under 16 years of age with positive SARS-CoV-2 nucleic acid test results admitted to hospital or managed by the Sydney Children's Hospital Network (SCHN) virtual care team. MAIN OUTCOME MEASURES: Age-specific SARS-CoV-2 infection frequency, overall and separately for SCHN virtual and hospital patients; rates of medical and social reason admissions, intensive care admissions, and paediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 per 100 SARS-CoV-2 infections; demographic and clinical factors that influenced likelihood of hospital admission. RESULTS: A total of 17 474 SARS-CoV-2 infections in children under 16 were recorded in NSW, of whom 11 985 (68.6%) received SCHN-coordinated care, including 459 admitted to SCHN hospitals: 165 for medical reasons (1.38 [95% CI, 1.17-1.59] per 100 infections), including 15 admitted to intensive care, and 294 (under 18 years of age) for social reasons (2.45 [95% CI, 2.18-2.73] per 100 infections). In an analysis that included all children admitted to hospital and a random sample of those managed by the virtual team, having another medical condition (adjusted odds ratio [aOR], 7.42; 95% CI, 3.08-19.3) was associated with increased likelihood of medical admission; in univariate analyses, non-asthmatic chronic respiratory disease was associated with greater (OR, 9.21; 95% CI, 1.61-174) and asthma/viral induced wheeze with lower likelihood of admission (OR, 0.38; 95% CI, 0.18-0.78). The likelihood of admission for medical reasons declined from infancy to 5-11 years, but rose again for those aged 12-15 years. Sex and Indigenous status did not influence the likelihood of admission. CONCLUSION: Most SARS-CoV-2 infections (Delta variant) in children were asymptomatic or associated with mild disease. Hospitalisation was relatively infrequent, and most common for infants, adolescents, and children with other medical conditions. More children were hospitalised for social than for medical reasons.

Home medication management problems and associated factors among psychiatric patients using home care pharmacy services at government hospitals in western Malaysia.

BACKGROUND: Proper home medication management plays a role in improving medication adherence, preserving drug efficacy and ensuring safe medication practices, which is crucial to establish positive treatment outcomes. However, no published studies are available on home medication management among psychiatric patients. The study aimed to identify home medication management problems among psychiatric patients in Malaysia and to examine the associations of inappropriate medication storage and lack of a medication administration schedule with sociodemographic factors, disease insight, number of medications and type of home care pharmacy services (HCPS). METHODS: This multicentre cross-sectional study was conducted among psychiatric patients using HCPS in six government hospitals in western Malaysia. Data were extracted from the HCPS form used for each visit as per the protocol published by the Pharmaceutical Services Division, Ministry of Health Malaysia. A minimum sample size of 169 was needed. Proportional random sampling was applied. The associations of inappropriate medication storage and lack of medication administration schedule with study parameters were analysed using multiple logistic regressions. RESULTS: A total of 205 home visits were conducted with 229 home medication management problems identified; inappropriate medication storage and lack of medication administration schedule topped the list. Inappropriate medication storage was significantly associated with low income [AOR = 4.34 (95% CI 1.17:15.98), p = 0.027], alcohol consumption [AOR = 14.26 (95% CI 1.82:111.38), p = 0.011], poor insight [AOR = 2.34 (95% CI 1.08:5.06), p = 0.030] and part-time HCPS [AOR = 2.60 (95% CI 1.20:5.67), p = 0.016]. Lack of administration schedule was significantly associated with low income [AOR = 6.90 (95% CI 1.46:32.48), p = 0.014], smoking [AOR = 2.43 (95% CI 1.20:4.92), p = 0.013], poor insight [AOR = 5.32 (95% CI 2.45:11.56), p < 0.05] and part-time HCPS [AOR = 2.96 (95% CI 1.42:6.15), p = 0.004]. CONCLUSIONS: Inappropriate medication storage and a lack of a medication administration schedule are common among psychiatric patients. The study also highlighted the potential of HCPS to improve disease insight and home medication management among psychiatric patients if the service is utilized fully.

The Effect of Maternal Self-Regulated Motivation on Breastfeeding Continuation.

OBJECTIVE: Women frequently report breastfeeding problems in the early postpartum period. Women who have self-endorsed beliefs that breastfeeding benefits their babies and themselves are more likely to continue breastfeeding despite breastfeeding barriers. Maternal self-endorsed beliefs is a key component of maternal self-regulated motivation. The present study examined the association between maternal self-regulated motivation, breastfeeding duration and exclusivity in Chinese women. METHODS: This was a prospective cohort study, of which we recruited participants in postnatal maternity units of publicly funded hospitals in Hong Kong. Postpartum women were asked to fill in the validated breastfeeding self-regulation questionnaire (BSRQ) before hospital discharge and their breastfeeding status was assessed by telephone follow-ups at 6 and 12 weeks postpartum. Multiple logistic regression was used to study the relationship between breastfeeding self-regulated motivation and the duration of breastfeeding at follow-up. RESULTS: At 6 and 12 weeks postpartum, women who breastfed exclusively scored significantly higher in self-regulated motivation than those who formula-fed. The self-regulated motivation was associated with higher odds of exclusive breastfeeding at 6 weeks and any breastfeeding at 12 weeks postpartum. CONCLUSIONS FOR PRACTICE: The study found that self-regulated motivation was positively related to breastfeeding duration. Maternal self-regulated motivation toward breastfeeding could be enhanced by the availability of social support and breastfeeding-friendly facilities, resulting in longer breastfeeding duration.

Prevalence of and factors associated with behavioral intention to take up home-based HIV self-testing among male clients of female sex workers in China - an application of the Theory of Planned Behavior.

ABSTRACTHIV self-testing (HIVST) is a useful means to increase coverage of HIV testing but under-utilized by male clients of female sex workers (MCFSW) in China. This study investigated the prevalence of and factors associated with behavioral intention to take up HIVST in the next year among MCFSW in Hong Kong, China. We recruited Chinse-speaking adult men who had patronized at least one female sex worker in the past six months. A total of 303 MCFSW completed a self-administered questionnaire. Univariate and multivariable ordinal logistic regression models were fit. Only 23.8% of participants indicated that they probably/definitely would take up HIVST in the next year. The multivariable ordinal logistic regression model showed that positive attitudes towards HIVST (adjusted odds ratios (aOR): 1.24, 95%CI: 1.13, 1.37), perceived HIVST uptake is completely under one's control (perceived behavioral control) (aOR: 1.44, 95%CI: 1.08, 1.92), and perceived higher risk of HIV infection (aOR: 1.32, 95%CI: 1.01, 1.76) were associated with higher intention to take up HIVST in the next year. The prevalence of behavioral intention to use HIVST was low among MCFSW in Hong Kong. Perceptions based on the Theory of Planned Behavior and perceived risk of HIV infection were associated with behavioral intention to use HIVST.

Factors impacting length of stay and survival in patients with advanced gynecologic malignancies and malignant bowel obstruction.

OBJECTIVES: Malignant bowel obstruction in patients with gynecologic malignancies can impose a large symptomatic burden. The objectives of this study were to identify factors associated with shorter length of hospital stay and overall survival in gynecologic oncology patients with malignant bowel obstructions. METHODS: A retrospective chart review was performed from December 2014 to March 2019 on patients admitted to a tertiary care center with a malignant bowel obstruction and advanced gynecologic malignancy. Data collection included patient and tumor characteristics, malignant bowel obstruction management (such as conservative management with bowel rest, nasogastric tube, pharmacotherapy or active intervention with surgery, chemotherapy, radiation, total parenteral nutrition or interventional stents), length of hospital stay, and survival outcomes. Statistical analysis included comparisons with Student's t-test and χ2 test, multivariable analysis, and survival analysis. RESULTS: A total of 107 patients with gynecologic cancer with malignant bowel obstruction were included. The majority of patients (63%, n=67) had ovarian cancer. The median length of hospital stay was 12 days (range 1-23), with a median overall survival after malignant bowel obstruction diagnosis of 7 months (range 0.1-64.1). Patients with active interventions had a longer length of stay compared with those with conservative management (13 vs 6 days, p<0.001). However, patients who received multiple active interventions had increased overall survival (9.1 vs 2.9 months, p=0.049). CONCLUSION: Patients who received multimodal treatment for malignant bowel obstruction had an increased length of stay and improvement in survival of over 6 months. This emphasizes the importance of a multidisciplinary approach to actively manage malignant bowel obstruction in advanced gynecologic cancer.

Disparities in access to palliative care facilities for patients with and without cancer: A retrospective review.

BACKGROUND: To date, little is known about the characteristics of patients who are admitted to a palliative care bed for end-of-life care. Previous data suggest that there are disparities in access to palliative care services based on age, sex, diagnosis, and socioeconomic status, but it is unclear whether these differences impact access to a palliative care bed. AIM: To better identify patient factors associated with the likelihood/rate of admission to a palliative care bed. DESIGN: A retrospective chart review of all initiated palliative care bed applications through an electronic referral program was conducted over a 24-month period. SETTING/PARTICIPANTS: Patients who apply and are admitted to a palliative care bed in a Canadian metropolitan city. RESULTS: A total of 2743 patients made a total of 5202 bed applications to 9 hospice/palliative care units in 2015-2016. Referred and admitted cancer patients were younger, male, and more functional than compared to non-cancer patients (all p < 0.001). Referred and admitted patients without cancer were more advanced in their illness trajectory, with an anticipated prognosis <1 month and Palliative Performance Status of 10%-20% (all p < 0.001). On multivariate analysis, a diagnosis of cancer and a prognosis of <3 months were associated with increased likelihood and/or rate of admission to a bed, whereas the presence of care needs, a longer prognosis and a PPS of 30%-40% were associated with decreased rates and/or likelihood of admission. CONCLUSION: Patients without cancer have reduced access to palliative care facilities at end-of-life compared to patients with cancer; at the time of their application and admission, they are "sicker" with very low performance status and poorer prognoses. Further studies investigating disease-specific clinical variables and support requirements may provide more insights into these observed disparities.

Trends in COVID-19 Testing and Positivity Rates from a Mobile Testing Program in the Phoenix Metropolitan Area.

The coronavirus (COVID-19) pandemic continues to be a global concern, with over 150 million cases worldwide. Arizona, which was recently a hotspot for COVID-19, has over 860 thousand cases. Reviewing the COVID-19 trends over time is crucial in understanding the pandemic and evaluating the impact of mitigation techniques. This current study analyzes the trends in COVID-19 testing and positivity rates from a mobile testing program in the Phoenix metropolitan area between December 2020 and April 2021. Of the 32,234 tests performed there was a total of 3654 COVID positive cases, yielding an overall positivity rate of 11.3%. COVID-19 positivity rates were significantly higher in December (12.9%) and January (12.7%), compared to February (7.5%), March (4.9%), and April (6.7%), p < 0.05. The peak of COVID-19 cases is likely attributable to the holiday season and family gatherings, followed by a steady decline, likely due to fewer gatherings and an increase in individuals receiving the COVID-19 vaccines. Continued public health measures, including vaccinations, are critical in reducing COVID-19 transmission.

COVID-19 Trends in the Phoenix Metropolitan Area from a Mobile Testing Program: Last Quarter of 2020.

The coronavirus (COVID-19) pandemic continues to be a public health concern, and Arizona has once again been a COVID-19 hotspot, peaking at 118.3 cases per 100,000. Understanding the trends in COVID-19 positivity rates over time is crucial in planning and mitigation of the virus. This current study analyzes the trends in COVID-19 testing, and COVID-19 antigen and antibody positivity rates over a 3-month time-span from October to December 2020. A retrospective study was conducted collecting data from a mobile testing program during October to December 2020 in the Phoenix metropolitan area. COVID-19 antigen and antibody positivity rates were analyzed. A total of 6710 patients were included in the study. As the months progressed, more patients were tested (October: 1635; November: 2037; December: 3038). The COVID-19 antigen positivity rate was significantly higher in December, compared to October and November (13.43% vs. 11.43 and 10.86%, p = 0.021). COVID-19 IgG rates were also significantly higher in November and December, compared to October (16.65 and 16.50% vs. 8.74%, p < 0.001). There was a progressive increase in COVID-19 positivity cases towards the end of 2020, likely attributed to factors including social gatherings during the holidays and the relaxing of the closure restrictions. Continued public health measures is crucial in preventing the spread of COVID-19.

Breastfeeding Duration and the Theory of Planned Behavior and Breastfeeding Self-Efficacy Framework: A Systematic Review of Observational Studies.

Introduction Numerous studies have shown that the constructs of the Theory of Reasoned Action (TRA), Theory of Planned Behavior (TPB) and Breastfeeding Self-Efficacy (BSE) Framework can effectively identify relationships between maternal psychosocial factors and breastfeeding initiation. However, the ability of these theories to predict breastfeeding duration has not been adequately analyzed. The aim of the review was to examine the utility of the constructs of TRA/TPB and BSE to predict breastfeeding duration. Methods We conducted a literature search using Pubmed (1980-May 2015), Medline (1966-May 2015), CINAHL (1980-May 2015), EMBASE (1980-May 2015) and PsycINFO (1980-May 2015). We selected studies that were observational studies without randomization or blinding, using TRA, TPB or BSE as the framework for analysis. Only studies reporting on breastfeeding duration were included. Results Thirty studies were selected, which include four using TRA, 10 using TPB, 15 using BSE and one using a combination of TPB and BSE. Maternal intention and breastfeeding self-efficacy were found to be important predictors of breastfeeding duration. Inconsistent findings were found in assessing the relationship between maternal attitudes, subjective norms, perceived behavior control and breastfeeding duration. Discussion The inadequacy of these constructs in explaining breastfeeding duration indicates a need to further explore the role of maternal self-determination in breastfeeding behavior.

Enhanced Recovery After Surgery Programs Improve Patient Outcomes and Recovery: A Meta-analysis.

INTRODUCTION: Enhanced recovery after surgery (ERAS) programs have been developed to improve patient outcomes, accelerate recovery after surgery, and reduce healthcare costs. ERAS programs are a multimodal approach, with interventions during all stages of care. This meta-analysis examines the impact of ERAS programs on patient outcomes and recovery. METHODS: A comprehensive search of all published randomized control trials (RCTs) assessing the use of ERAS programs in surgical patients was conducted. Outcomes analyzed were length of stay (LOS), overall mortality, 30-day readmission rates, total costs, total complications, time to first flatus, and time to first bowel movement. RESULTS: Forty-two RCTs involving 5241 patients were analyzed. ERAS programs significantly reduced LOS, total complications, and total costs across all types of surgeries (p < 0.001). Return of gastrointestinal (GI) function was also significantly improved, as measured by earlier time to first flatus and time to first bowel movement, p < 0.001. There was no overall difference in mortality or 30-day readmission rates; however, 30-day readmission rates after upper GI surgeries nearly doubled with the use of ERAS programs (RR = 1.922; p = 0.019). CONCLUSIONS: ERAS programs are associated with a significant reduction in LOS, total complications, total costs, as well as earlier return of GI function. Overall mortality and readmission rates remained similar, but there was a significant increase in 30-day readmission rates after upper GI surgeries. ERAS programs are effective and a valuable part in improving patient outcomes and accelerating recovery after surgery.

Medical student attitudes and educational interventions to prevent neurophobia: a longitudinal study.

BACKGROUND: With an aging American population, the burden of neurologic disease is intensifying and the decline in neurology residents and practicing neurologists is leaving these patients helpless and unable to find care. 'Neurophobia', a chronic illness that begins early in medical school, has been identified as a cause for the low number of neurology residents. METHODS: A longitudinal study surveyed medical students at the beginning of their first year (M1) and then again at the beginning of their second year (M2). Three neuroscience educational interventions were studied: team based learning (TBL), case based teaching (CBT), and problem based learning (PBL). Participants provided self-reported neurophobia levels, attitudes about neuroscience, and the effectiveness of educational interventions. RESULTS: A total of 446 students during M1 and 206 students during M2 participated in the survey. A significant change in self-reported neurophobia (p = 0.035) was observed from 19% in M1 to 26% in M2. Neuroscience knowledge and confidence managing a neurologic condition also significantly increased (p < 0.001 and p = 0.038 respectively). Perceived interest, difficulty, and desire to pursue a career in neuroscience did not a change significantly. Majority of students perceived CBT (76%), TBL (56%), and PBL (66%) beneficial. Only CBT demonstrated a statistical difference (p = 0.026) when stratified by self-reported change in neurophobia. CONCLUSION: An increase in neurophobia after completing a neuroscience was observed but the prevalence rate of 26% was lower than previous studies. Knowledge about neuroscience increased significantly and educational interventions were considered beneficial by students. Thus, interventions that increase knowledge and decrease neurophobia can lead to an increase in students pursuing neurology residencies.

Ultrasound-guided central venous catheter placement increases success rates in pediatric patients: a meta-analysis.

BACKGROUND: Real-time ultrasound (US) guidance for central venous catheter (CVC) insertion has been shown to increase cannulation success rates and reduce complications in adults. Literature regarding US-guided CVC placement in children remains limited and conflicting. This meta-analysis examines the efficacy and safety of US-guided CVC placement among pediatric patients. METHODS: A comprehensive literature search of all published randomized control trials (RCTs) comparing the use of real-time US-guided CVC insertion with anatomic landmark (LM)-guided CVC insertion in pediatric patients <18 y of age was conducted. Outcomes analyzed were cannulation success rate, number of attempts required, incidence of accidental arterial puncture, and time to cannulation. RESULTS: Eight RCTs involving 760 patients were analyzed. US-guided CVC insertion significantly increased success rates by 31.8% and decreased the mean number of attempts required. A trend toward a decrease in the risk of accidental arterial puncture with the use of US-guided CVC insertion was also observed. US-guided CVC insertion was not associated with a significant difference in time required for CVC placement. CONCLUSION: US-guided CVC placement is associated with significantly higher success rates and decreased mean number of attempts required for cannulation. US-guided CVC insertion improves success rates, and should be utilized in pediatric patients.

Pediatric Chordomas: A Population-Based Clinical Outcome Study Involving 86 Patients from the Surveillance, Epidemiology, and End Result (SEER) Database (1973-2011).

BACKGROUND/AIMS: Primary chordomas, rare cancers arising from the notochord remnants, are extremely rare in the pediatric population. This study examined a large cohort of primary chordoma patients to determine factors impacting prognosis and survival. METHODS: Demographic and clinical data on 1,358 primary chordoma patients (86 pediatric patients ≤19 years of age and 1,272 adult patients ≥20 years of age) were abstracted from the Surveillance, Epidemiology, and End Result (SEER) database (1973-2011). RESULTS: Pediatric primary chordomas present most often as small tumors <4 cm in the cranium of male Caucasians. Despite the majority of primary chordomas presenting with locoregional involvement (90.4%), pediatric patients had more distant disease (14.8 vs. 9.2%, p < 0.05). Survival among pediatric patients having surgery only was significantly longer than for adults (22.5 vs. 14.3 years, p < 0.001). Overall survival was longer (17.2 vs. 12.6 years) and overall mortality was lower in pediatric patients (38.4 vs. 49.8%), but cancer-specific mortality was higher (37.2 vs. 28.6%, p < 0.005). CONCLUSIONS: Pediatric primary chordomas present most often as small tumors <4 cm in the cranium of male Caucasians. Despite having a higher rate of metastasis, they have prolonged survival compared to adults. Surgical resection significantly improves survival in pediatric primary chordoma patients, and should be considered as first-line therapy in all eligible children.