Publications by authors named "Benjamin Robert"

this study aims to evaluate the survival outcomes of patients suffering from head and neck synovial sarcoma (HNSS), especially in relation to patients with a localized disease at diagnosis. this retrospective chart review includes 57 patients diagnosed with primary HNSS between 1981 and 2020 who presented with a localized disease at diagnosis. Overall survival (OS) from diagnosis, local recurrence-free survival (LRFS), and metastasis-free survival (MFS) from the end of the primary tumor treatment are estimated.

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  • The study aimed to assess how baseline imaging parameters from F-18-fluorodeoxy-D-glucose PET/CT can predict treatment responses in patients with dedifferentiated and well-differentiated liposarcomas.
  • A total of 24 patients were evaluated, with specific parameters like SUVmax, SUVmean, and Hounsfield units calculated from their PET and CT images to measure tumor response to systemic therapy.
  • The research identified that new baseline SUV/HU ratios were effective indicators of treatment outcomes, suggesting changes in SUVmean may provide better insights than traditional metrics alone.
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Objective: We evaluated survival outcomes by primary tumor site in synovial sarcoma (SS) patients with localized and metastatic disease at diagnosis.

Methods: We conducted a retrospective review of 504 SS patients diagnosed from 1974 to 2020. Kaplan-Meier method, log-rank test, and Cox-proportional hazards regression were used.

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Background: Moderately hypofractionated, preoperative radiotherapy in patients with soft tissue sarcomas (HYPORT-STS; ClinicalTrials.gov identifier NCT03819985) investigated a radiobiologically equivalent, moderately hypofractionated course of preoperative radiotherapy (RT) 15 × 2.85 Gy in patients with soft tissue sarcoma (STS).

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Rhabdomyosarcomas are the most common soft tissue sarcoma in children, and pediatric alveolar rhabdomyosarcoma (ARMS) prognosis has improved based on cooperative studies. However, in adults, ARMS is significantly rarer, has poorer outcomes, and currently lacks optimal treatment strategies. This study aimed to evaluate the clinical outcome of an adult ARMS population with different front-line systemic chemotherapies and determine if any chemotherapy regimen is associated with improved survival.

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Efforts to unveil the structure of the local interstellar medium and its recent star-formation history have spanned the past 70 years (refs. ). Recent studies using precise data from space astrometry missions have revealed nearby, newly formed star clusters with connected origins.

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Background: Undifferentiated pleomorphic sarcomas (UPSs) are amongst the most common subtypes of soft-tissue sarcomas. Few real-world data on the use of immune checkpoint blockade (ICB) in UPS patients and other high-grade pleomorphic STS patients are available.

Purpose: The purpose of our study is to describe the efficacy and toxicity of ICB in patients with advanced UPSs and other high-grade pleomorphic sarcomas treated at our institution.

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Aim: Despite advances in diabetes treatments, youth commonly fail to meet glucose targets. Telehealth support may help youth meet diabetes related goals. The objective of the project was to assess whether intensive telehealth support in a group of poorly controlled youth with diabetes would help improve glycated hemoglobin (HbA1c) levels and decrease hospitalization rates over a 12-month time frame.

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Ripretinib and avapritinib have demonstrated activity in the late-line treatment of gastrointestinal stomal tumors (GISTs). We investigated whether patients previously treated with ripretinib benefit from avapritinib, and vice versa. Patients diagnosed with metastatic/unresectable GIST and treated with both drugs at two institutions in 2000-2021 were included.

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  • A randomized phase 2 trial was conducted to evaluate the effectiveness of neoadjuvant immune checkpoint blockade (ICB) therapies (nivolumab and nivolumab/ipilimumab) in patients with resectable retroperitoneal DDLPS and extremity/truncal UPS.
  • The primary endpoint of pathologic response showed a median hyalinization of 8.8% in DDLPS and 89% in UPS, with secondary endpoints focusing on immune changes and survival rates over time.
  • Results indicated that lower pre-treatment regulatory T cell densities correlated with better pathological outcomes, and that neoadjuvant ICB led to significant immune changes and benefits, particularly in patients with UPS when combined with radiation therapy
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Objectives: The purpose of this study was to evaluate a three-material decomposition algorithm for hepatic fat quantification using a dual-layer computed tomography (DL-CT) and MRI as reference standard on a large patient cohort.

Method: A total of 104 patients were retrospectively included in our study, i.e.

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  • Hyperglycaemia in Type 1 diabetes (T1D) is mainly due to a lack of insulin, but insulin resistance (IR) can also worsen blood sugar instability and lead to long-term heart issues.
  • A study examined blood samples from 28 children with T1D to identify how certain metabolites relate to blood sugar control and IR, using statistical analyses to find significant correlations.
  • Results indicated that incomplete fat oxidation and the way fats and amino acids are metabolized are linked to blood sugar control and insulin dosage in young children with T1D.
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Glycogen storage disease Ia (GSD Ia), also known as von Gierke disease, is caused by pathogenic variants in the gene (OMIM 232200) which encodes glucose-6-phosphatase. Deficiency of glucose-6-phosphatase impairs the processes of gluconeogenesis and glycogenolysis by preventing conversion of glucose-6-phosphate to glucose. Clinical features include fasting hypoglycemia, lactic acidosis, hypertriglyceridemia, hyperuricemia, hepatomegaly, and development of hepatocellular adenomas (HCAs) with potential for malignant transformation.

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Context: Increases in incident cases of pediatric type 1 (T1D) and type 2 diabetes (T2D) were observed during the first year of the COVID-19 pandemic.

Objective: This work aimed to identify trends in incidence and presentation of pediatric new-onset T1D and T2D during the second year of the COVID-19 pandemic.

Methods: A retrospective chart review was conducted.

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Purpose: Developing new therapeutics for any of the more than 100 sarcoma subtypes presents a challenge. After progression from standard therapies, patients with sarcoma may be referred for enrollment in early-phase trials. This study aimed to investigate whether enrollment in biomarker-matched early-phase clinical trials leads to better outcomes for patients with advanced sarcoma.

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Objective: Our objective was to determine the prevalence of insulin treatment in premature infants with hyperglycemia and evaluate the association of length of treatment with outcomes.

Study Design: The study included cohort of 29,974 infants 22 to 32 weeks gestational age (GA) admitted to over 300 neonatal intensive care unit (NICU) from 1997 to 2018 and diagnosed with hyperglycemia.

Results: Use of insulin significantly decreased during the study period ( = 0.

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  • The study focuses on evaluating the safety of a shorter, moderately hypofractionated radiotherapy regimen (42.75 Gy over 15 fractions) for patients with non-metastatic soft tissue sarcomas, compared to the standard regimen of 50 Gy over 25 fractions.
  • Conducted at MD Anderson Cancer Center, the trial aimed to determine if the new regimen would result in fewer major wound complications within 120 days post-surgery, which historically affect about 35% of patients.
  • A total of 120 patients were enrolled from December 2018 to January 2021, and the trial has been registered for ongoing follow-up to assess safety and outcomes.
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  • Undifferentiated pleomorphic sarcoma (UPS) shows significant immune infiltration, with immune checkpoint inhibitors benefiting around 20% of patients, leading to an investigation of the tumor's immune microenvironment and its impact on patient outcomes.
  • Researchers performed immunohistochemistry on 105 surgically removed UPS samples, assessing various immune markers and correlations related to overall and disease-free survival using statistical methods.
  • Key findings revealed that certain immune markers (like CD39 and CD73) were linked to treatment responsiveness and survival rates, with significant variations seen in primary vs. recurrent and metastatic tumors, highlighting the complexity of immune interactions in UPS.
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  • - The Connective Tissue Oncology Society established guidelines for identifying ultrarare sarcomas (URS), which occur in fewer than 1 in 1,000,000 people; a study analyzed 56 patients with soft tissue sarcomas and 21 with bone sarcomas from Phase 1 trials.
  • - Out of 587 sarcoma cases, 18.1% were URS, predominantly affecting males with a median age of 44.3, and they showed a higher objective response rate to treatment (13.2%) compared to common sarcomas (6.9%).
  • - The study highlights that matched trials based on genomic profiling resulted in better outcomes, with a median overall survival of 27.
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  • Seizure clusters, defined as two or more seizures in a six-hour period, are prevalent in pediatric epilepsy patients, with about one-third of participants experiencing them during a year-long study.
  • The study classified patients into high, intermediate, and low-risk groups based on their seizure history, finding that those with more frequent seizures were more likely to experience clusters.
  • Despite identifying at-risk factors and the use of rescue medications, these medications were underutilized, suggesting a need for future research on their effectiveness in managing seizure clusters and preventing related complications.
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Objective: Well-differentiated (WDLPS) and dedifferentiated liposarcoma (DDLPS) account for the majority of liposarcomas. Although gemcitabine-docetaxel is used as second-line treatment in soft tissue sarcomas, its efficacy in WDLPS/DDLPS is not established. This study retrospectively analyzed the efficacy of gemcitabine regimens in WDLPS/DDLPS.

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Background: In ultra-rare sarcomas (URS) the conduction of prospective, randomized trials is challenging. Data from retrospective observational studies (ROS) may represent the best evidence available. ROS implicit limitations led to poor acceptance by the scientific community and regulatory authorities.

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