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.

     

La pandemia COVID-19 y su repercusión en la unidad de radiología intervencionista: nuestra experiencia

This paper aims to describe our experience in an interventional radiology unit in a hospital in Spain that was severely affected by the COVID-19 pandemic. To this end, we did a prospective observational study of 20 consecutive patients with COVID-19 who underwent 21 interventional radiology procedures between March 13, 2020 and May 11, 2020. We describe the measures taken to reorganize the work and protective measures, as well as the repercussions of the situation on our unit's overall activity and activity in different phases. The COVID-19 pandemic has represented a challenge in our daily work, but learning from our own experience and the recommendations of the Spanish radiological societies (SERVEI and SERAM) has enabled us to adapt successfully. Our activity dropped only 22% compared to the same period in 2019.     

Automatizing a nonscripting TPS for optimizing clinical workflow and reoptimizing IMRT/VMAT plans

The purpose of this study was to design a toolkit that interacts with the Monaco (Elekta AB, Stockholm, Sweden) treatment planning system (TPS) for optimization of intensity-modulated radiation therapy and volumetric-modulated arc therapy without the need for a dedicated application programming interface. Successful inverse planning of radiotherapeutic treatment depends on the tweaking of many parameters; a tool was thus needed to explore these parameters more exhaustively without significantly increasing planning time. The software that we used was based on an open-source library that mimics human interaction with Microsoft Windows applications. We developed a simple Autoflow software routine that analyzes and optimizes calculated plans by considering the relative impact of different cost functions and modifying constraints accordingly. It was also designed to change segmentation parameters to fit more complex treatments. The toolkit is publicly available for download at https://bitbucket.org/hgugmradiofisica/pymonaco/src/master/. A study of prostate cancer cases was conducted to compare automatically created plans with previously treated cases. The toolkit fully automated the radiotherapy planning procedure, allowing the TPS to calculate or optimize plans during nonworking hours. In the prostate study, the use of this tool reduced the dose to organs at risk with a negligible decrease in target coverage. This tool enables the efficient use of the TPS, allowing research and clinical applications to coexist without conflict. It provides consistency and efficiency throughout the treatment planning process, which may be of great value to clinics with few resources. The impact of this tool on clinical workflow is important, as it not only provides better efficiency, but also increases treatment quality.     

Analysis of KCNH2 and CACNA1C schizophrenia risk genes on EEG functional network modulation during an auditory odd-ball task

European Archives of Psychiatry and Clinical Neuroscience - A deficit in task-related functional connectivity modulation from electroencephalogram (EEG) has been described in schizophrenia. The use...     

Deficits of entropy modulation of the EEG: A biomarker for altered function in schizophrenia and bipolar disorder?

BackgroundThe synchronized activity of distributed neural assemblies — reflected in the electroencephalogram (EEG) — underpins mental function. In schizophrenia, modulation deficits of EEG spectral content during a P300 task have been replicated. The effects of treatment, chronicity and specificity in these deficits and their possible relationship with anatomic connectivity remain to be explored.MethodsWe assessed spectral entropy modulation of the EEG during a P300 task in 79 patients with schizophrenia (of those, 31 were in their first episode), 29 patients with bipolar disorder and 48 healthy controls. Spectral entropy values summarize EEG characteristics by quantifying the irregularity of spectral content. In a subsample, we calculated the network architecture of structural connectivity using diffusion tensor imaging and graph-theory parameters.ResultsWe found significant spectral entropy modulation deficits with task performance in patients with chronic or first-episode schizophrenia and in patients with bipolar disorder, without significant pre-stimulus spectral entropy differences. The deficits were unrelated to treatment doses, and spectral entropy modulation did not differ between patients taking or not taking antipsychotics, lithium, benzodiazepines or antidepressants. Structural connectivity values were unrelated to spectral entropy modulation. In patients with schizophrenia, spectral entropy modulation was inversely related to negative symptoms and directly related to verbal memory.LimitationsAll patients were taking medication. Patients with bipolar disorder were euthymic and chronic. The cross-sectional nature of this study prevented a more thorough analysis of state versus trait criteria for spectral entropy changes.ConclusionSpectral entropy modulation with task performance is decreased in patients with schizophrenia and bipolar disorder. This deficit was not an effect of psychopharmacological treatment or structural connectivity and might reflect a deficit in the synchronization of the neural assemblies that underlie cognitive activity.