Metastatic adult-type non-rhabdomyosarcoma soft tissue sarcomas in children and adolescents: A cohort study from the European paediatric Soft tissue sarcoma Study Group

Background

Limited data exist on the clinical behavior of pediatric non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) with distant metastases at onset, and a clear standard of care has not yet been defined.

Methods

This cohort study reports on pediatric adult-type metastatic NRSTS enrolled in two concurrent prospective European studies, i.e., the randomized BERNIE study and the single-arm MTS 2008 study developed by the European paediatric Soft tissue sarcoma Study Group. Treatment programs were originally designed for patients with metastatic rhabdomyosarcoma, i.e., nine courses of multidrug chemotherapy (with or without bevacizumab in the BERNIE study), followed by 12 cycles of maintenance therapy, whereas radiotherapy and/or surgery (on primary tumor and/or metastases) were delayed until after seven courses of chemotherapy had been administered.

Results

The study included 61 patients <21 years old treated from July 2008 to December 2016. The lung was the site of metastases in 75% of the cases. All patients received multi-agent chemotherapy, 44% had local therapy to primary tumor, and 18% had treatment of metastases. Median time to progression/relapse was 6 months. A high rate of tumor progression was observed during the initial part of the chemotherapy program. With a median follow-up of 41.5 months (range, 2–111 months), 3-year event-free survival and overall survival were 15.4% (95% confidence interval [CI], 7.6–25.7) and 34.9% (95% CI, 22.7–47.5), respectively. There were no statistically significant differences in outcome depending on the type of treatment administered.

Conclusions

The study confirmed the overall poor outcome for patients with metastatic NRSTS, whose treatment remains a challenge.

Plain Language Summary

• Pediatric non-rhabdomyosarcoma soft tissue sarcomas form a heterogeneous group of rare tumors.
• Although recent international studies have defined the standard of care for patients with localized disease, limited data are available on the clinical behavior of patients with distant metastases.
• This study on 61 metastatic cases treated on two prospective European protocols confirms that the chances of survival of such patients are often dismal and a standard treatment is still lacking.

     

Sex and smoking differences in the association between gastroesophageal reflux and risk of esophageal squamous cell carcinoma in a high-incidence area: Golestan Cohort Study

Prior studies have conflicting findings regarding the association between gastroesophageal reflux disease (GERD) and esophageal squamous cell carcinoma (ESCC). We examined this relationship in a prospective cohort in a region of high ESCC incidence. Baseline exposure data were collected from 50 045 individuals using in-person interviews at the time of cohort entry. Participants were followed until they developed cancer, died, or were lost to follow up. Participants with GERD symptoms were categorized into any GERD (heartburn or regurgitation), mixed symptoms, or heartburn alone. Multivariable Cox regression was used to assess the relationship between GERD symptom group and histologically confirmed ESCC. The model was adjusted for known risk factors for GERD and ESCC. 49 559 individuals were included in this study, of which 9005 had GERD symptoms. Over 13.0 years of median follow up, 290 individuals were diagnosed with ESCC. We found no association between any GERD and risk of ESCC (aHR 0.90, 95% CI: 0.66-1.24, P = .54). Similar findings were observed for the GERD symptom subtypes. Significant interactions between any GERD and sex (P = .013) as well as tobacco smoking (P = .028) were observed. In post-hoc analyses, GERD was associated with a decreased risk of ESCC in men (aHR 0.51, 95% CI: 0.27-0.98 P = .04) and in smokers (aHR 0.26, 95% CI: 0.08-0.83 P = .02). While there was little evidence for an overall association between GERD symptoms and ESCC risk, significant interactions with sex and smoking were observed. Men and smokers with GERD symptoms had a lower risk of ESCC development.     

Validation of a Modified Jefferson Scale of Empathy for Observers to Assess Trainees

Objective“Demonstrate insight and understanding into emotion” is a competency amenable to simulation-based assessment. The Jefferson Scale of Patient Perceptions of Physician Empathy (JSPPPE) has validity evidence for patients to assess provider empathy. A version adapted for a third-party observers does not exist. Our aim was to modify the JSPPPE and use recorded standardized encounters to obtain validity evidence.MethodsThis cross-sectional study used video and data collected from 2 pediatric residencies. In 2018, 4 raters reviewed 24 videos of 12 interns communicating with standardized patients (SP) in 2 encounters and completed a modified JSPPE for observers (JSEO). Reliability between raters was established using Intraclass Correlations (ICC). JSEO mean scores were correlated to Essential Elements of Communication (EEC), JSPPPE, and faculty composite interpersonal communication (IC) scores using Spearman Rank.ResultsThe mean ICC for all 4 raters was 0.573 (0.376–0.755). When ICC was calculated for pairs of raters, Rater 1 was an outlier. ICCs for mean scores for pairs among the 3 remaining raters was 0.81 to 0.84. Mean JSEO scores from the four raters correlated with the JSPPPE (rho = 0.45, P = .03) and IC (rho = 0.68, P < .001), but not the EEC (rho = 0.345, P = .1).ConclusionsWe found validity evidence for the use of a modified JSPPPE for an observer to assess empathy in a recorded encounter with a SP. This may be useful as medical educators shift toward competency-based tracking. The brevity of this tool and potential assessment using video are also appealing.     

The effect of caffeine abstinence on sleep among habitual caffeine users with poor sleep

Caffeine is the most widely used psychoactive substance in the world and is known to disrupt healthy sleep. However, very few studies have directly tested the effect of caffeine abstinence on sleep, and these have yielded inconsistent findings. The purpose of the present study was to examine changes in sleep following caffeine abstinence and examine the extent to which characteristics of habitual caffeine use moderated this change. Participants included 66 healthy, young adults with habitual caffeine use and poor sleep. During the 2‐week baseline, sleep was assessed using wrist actigraphy and daily caffeine use was assessed with bedtime diaries. Eligible participants then completed 1 week of caffeine abstinence, during which sleep was measured with wrist actigraphy. Multilevel models found no significant differences between either mean levels or growth trajectories of total sleep time or sleep efficiency between baseline and caffeine abstinence. Mean levels of sleep onset latency also did not differ between baseline and caffeine abstinence. A small but significant quadratic effect was observed, such that sleep onset latency decreased during the first few days of caffeine abstinence, then increased to levels above baseline. Characteristics of caffeine use did not moderate changes in sleep between baseline and caffeine abstinence. These data suggest that abstaining from caffeine may not result in long‐term sleep improvement for habitual caffeine users, which contradicts the common sleep health recommendation. The present findings encourage more rigorous investigation of the effectiveness of caffeine restriction on sleep.     

Mental health disorder,pain, and pain treatment among long-term care residents: Evidence from the Minimum Data Set 3.0

Objective: This study evaluated: (a) associations between long-term care residents’ mental health disorder diagnoses and their pain self-reports and pain treatments, and (b) the extent to which communication, cognitive, and physical functioning problems help explain disparities in the pain and pain treatments of long-term care residents with and without mental health disorders.

Method: Minimum Data Set 3.0 records of 8,300 residents of Department of Veterans Affairs Community Living Centers were used to determine statistically unadjusted and adjusted cross-sectional associations between residents' mental health diagnoses and their pain and pain treatments.

Results: Residents diagnosed with dementia and serious mental illness (SMI) were less likely, and those diagnosed with depressive disorder, post-traumatic stress disorder (PTSD), and substance use disorder (SUD) were more likely, to report recent, severe, and debilitating pain. Among residents affirming recent pain, those with dementia or SMI diagnoses were twice as likely to obtain no treatment for their pain and significantly less likely to receive as-needed pain medication and non-pharmacological pain treatments than were other residents. Those with either depressive disorder or PTSD were more likely, and those with SUD less likely, to obtain scheduled pain medication. In general, these associations remained even after statistically adjusting for residents' demographic characteristics, other mental health disorder diagnoses, and functioning.

Conclusion: Long-term care residents with mental health disorders experience disparities in pain and pain treatment that are not well-explained by their functioning deficits. They may benefit from more frequent, thorough pain assessments and from more varied and closely tailored pain treatment approaches.