Publications by authors named "Robin Jones"

Objectives: Gastrointestinal stromal tumors, the most prevalent mesenchymal tumors (80 %) of the gastrointestinal tract, comprise less than 1 % of all gastrointestinal neoplasms and about 5 % of all sarcomas. Despite their rarity, Gastrointestinal stromal tumors present diverse clinical manifestations, anatomic locations, histological subtypes, and prognostic outcomes.

Methods: This scoping review comprehensively explores the epidemiology, clinical characteristics, diagnostic and prognostic modalities, as well as new therapeutic options for Gastrointestinal stromal tumors.

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  • The treatment of advanced chondrosarcoma, which often doesn't respond well to standard chemotherapy, is currently being explored through targeted therapies aimed at specific genetic mutations and signaling pathways.
  • New strategies include utilizing IDH inhibitors for tumors with IDH mutations, hedgehog pathway inhibitors, and various forms of immunotherapy, all facing challenges but showing potential for effectiveness.
  • There's a strong emphasis on using personalized, multi-omics approaches to create combination therapies that can better address the heterogeneous nature of chondrosarcoma and possibly improve treatment outcomes.
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  • Drug-induced stuttering is an acquired speech disorder caused by certain medications, resembling developmental stuttering, and has been primarily studied through case reports and adverse drug reactions.
  • A recent study analyzed electronic health records from a major medical center to identify and classify drugs linked to this type of stuttering, reviewing 40 suspected cases.
  • The findings revealed that 18 different drugs were associated with stuttering in 22 individuals, especially in the classes of antiseizure agents, CNS stimulants, and antidepressants, with topiramate being the most commonly implicated drug; the study emphasizes the need for better documentation of medication-related speech issues in EHRs.
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We present the case of a patient with Myxofibrosarcoma (MFS), a mesenchymal type of soft tissue sarcoma (STS) and the response to combination immunotherapy with anti PD-1 and anti-CTLA-4 therapy, following disease progression after Standard chemotherapy (SACT) and Radiotherapy (RT). We have shown a timeline of treatment and responses, as well as the overall safety profile and the management of immunotherapy related adverse events. This study demonstrates the potential of checkpoint inhibitors as therapeutic agents in the treatment of MFS.

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The aim of the study was to report the outcome of primary localized low-grade fibromyxoid sarcoma (LGFMS), sclerosing epithelioid fibrosarcoma (SEF), and hybrid LGFMS/SEF (H-LGFMS/SEF). Patients with primary localized LGFMS, SEF, or H-LGFMS/SEF, surgically treated with curative intent from January 2000 to September 2022, were enrolled from 14 countries and 27 institutions. Pathologic inclusion criteria were predefined by expert pathologists.

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  • Epithelioid hemangioendothelioma (EHE) is difficult to treat with traditional chemotherapy, prompting researchers to explore new therapies such as sirolimus and identify biomarkers for tumor aggressiveness.
  • Scientists created a patient-derived xenograft (PDX) model from an advanced EHE patient to test sirolimus and to study serum levels of Growth/Differentiation Factor 15 (GDF-15) as a potential biomarker.
  • The results indicated sirolimus was more effective than doxorubicin in reducing tumor growth and GDF-15 levels, establishing GDF-15 as a promising biomarker for EHE aggressiveness and potentially indicating the effectiveness of sirolimus in patients.
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Somatic activating mutations drive most gastrointestinal stromal tumors (GISTs). Disease progression eventually develops with first-line imatinib, commonly due to secondary mutations, and different kinase inhibitors have various levels of treatment efficacy dependent on specific acquired resistance mutations. Ripretinib is a broad-spectrum switch-control KIT/PDGFRA tyrosine kinase inhibitor for patients with advanced GIST who received prior treatment with three or more kinase inhibitors, including imatinib.

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  • Epithelioid hemangioendothelioma (EHE) is a rare type of cancer with unique features, but its natural history and best treatment practices are not well understood.
  • The EURACAN project has created a registry to gather prospective data on newly diagnosed EHE patients to enhance understanding of the disease.
  • The study will involve collecting comprehensive patient data from specialized hospitals to identify prognostic factors, treatment efficacy, and to monitor the disease's progression and outcomes over time.
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  • Soft tissue sarcomas make up a rare group of tumors accounting for just 1% of solid cancers, with liposarcoma being a notable subtype.
  • The study focused on 21 patients with atypical lipomatous tumors/well differentiated liposarcoma (ALT/WDLPS) and dedifferentiated liposarcoma (DDLPS), investigating the role of biomarkers CDK4 and MDM2.
  • Results indicated that CDK4 was more highly expressed in DDLPS than in ALT/WDLPS, and the CDK4 inhibitor palbociclib showed promise in a sequential treatment approach when combined with lenvatinib, suggesting a new management strategy for DDLPS.
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The optimal medical treatment of chemotherapy-ineligible patients affected by advanced soft-tissue sarcomas is unclear. In this population, tyrosine kinase inhibitors represent an appealing alternative treatment strategy. First-line use of the tyrosine kinase inhibitor anlotinib in chemotherapy-ineligible patients with soft-tissue sarcoma showed promising activity across multiple histologies.

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Soft tissue sarcomas (STS) are a rare and heterogeneous group of cancers. Treatment options have changed little in the past thirty years, and the role of neoadjuvant chemotherapy is controversial. Accurate risk stratification is crucial in STS in order to facilitate clinical discussions around peri-operative treatment.

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Background: High-risk soft tissue sarcomas of the extremities and trunk wall (eSTS), as defined by the Sarculator nomogram, are more likely to benefit from (neo)adjuvant anthracycline-based therapy compared to low/intermediate-risk patients. The biology underpinning these differential treatment outcomes remain unknown.

Methods: We analysed proteomic profiles and clinical outcomes of 123 eSTS patients.

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A "Think Tank for Osteosarcoma" medical advisory board meeting was held in Santa Monica, CA, USA on February 2-3, 2024. The goal was to develop a strategic approach to prevent recurrence of osteosarcoma. Osteosarcoma metabolism and the genomic instability of osteosarcoma, immunotherapy for osteosarcoma, CAR-T cell therapy, DeltaRex-G tumor-targeted gene therapy, repurposed drugs, alternative medicines, and personalized medicine were discussed.

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Purpose: One manifestation of systemic inequities in communication sciences and disorders (CSD) is the chronic underreporting and underrepresentation of sex, gender, race, and ethnicity in research. The present study characterized recent demographic reporting practices and representation of participants across CSD research.

Methods: We systematically reviewed and extracted key reporting and participant data from empirical studies conducted in the United States (US) with human participants published in the year 2020 in journals by the American Speech-Language-Hearing Association (ASHA; = 407 articles comprising a total = 80,058 research participants, search completed November 2021).

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Background: British Sarcoma Group guidelines for the management of GIST were initially informed by those published by the European Society of Clinical Oncology. This update was written by a group of experts to includes a discussion of the highlight improvements in our knowledge of the disease and recent treatment developments. The guidelines include sections on Incidence, Aetiology, Diagnosis, including risk assessment, Treatment and Follow-up.

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Purpose: The landscape of extracellular matrix (ECM) alterations in soft tissue sarcomas (STS) remains poorly characterized. We aimed to investigate the tumor ECM and adhesion signaling networks present in STS and their clinical implications.

Experimental Design: Proteomic and clinical data from 321 patients across 11 histological subtypes were analyzed to define ECM and integrin adhesion networks.

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The management of sarcomas in specialist centers delivers significant benefits. In much of the world, specialists are not available, and the development of expertise is identified as a major need. However, the terms 'specialist' or 'expert' center are rarely defined.

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Background: Adolescents and young adult (AYA) patients with soft tissue tumours including sarcomas are an underserved group with disparities in treatment outcomes.

Methods: To define the molecular features between AYA and older adult (OA) patients, we analysed the proteomic profiles of a large cohort of soft tissue tumours across 10 histological subtypes (AYA n = 66, OA n = 243), and also analysed publicly available functional genomic data from soft tissue tumour cell lines (AYA n = 5, OA n = 8).

Results: Biological hallmarks analysis demonstrates that OA tumours are significantly enriched in MYC targets compared to AYA tumours.

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People of all ages consume salt every day, but is it really just salt? Plastic nanoparticles [nanoplastics (NPs)] pose an increasing environmental threat and have begun to contaminate everyday salt in consumer goods. Herein, we developed a combined surface enhanced Raman scattering (SERS) and stimulated Raman scattering (SRS) approach that can realize the filtration, enrichment, and detection of NPs in commercial salt. The Au-loaded (50 nm) anodic alumina oxide substrate was used as the SERS substrate to explore the potential types of NP contaminants in salts.

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  • Leiomyosarcoma (LMS) is a common type of soft tissue sarcoma, making up about 20% of cases, with varying survival rates influenced by anatomical differences, recurrence risks, and histological variants.
  • Current treatment approaches are too generalized, highlighting the need for personalized strategies based on individual patient characteristics and genomic features discovered through tissue profiling.
  • To enhance patient outcomes, it's essential to collaborate on a larger scale, pooling expertise and resources to improve risk stratification and develop biomarker-matched therapies for LMS.
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Purpose: This exploratory analysis evaluated the tumor samples of the patients treated with doxorubicin (with or without olaratumab) in a negative phase III ANNOUNCE trial to better understand the complexity of advanced soft tissue sarcomas (STS) and to potentially identify its predictive markers.

Experimental Design: RNA sequencing was performed on pretreatment tumor samples (n = 273) from the ANNOUNCE trial to evaluate response patterns and identify potential predictive treatment markers for doxorubicin. A BOR-associated signature to doxorubicin (REDSARC) was created by evaluating tumors with radiographic response versus progression.

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Ripretinib, a novel tyrosine kinase inhibitor used in advanced gastrointestinal stromal tumors (GIST) resistant to standard therapies, was assessed in the United Kingdom (UK) within an Expanded Access Program (EAP). A retrospective review of patients treated between January 2020 and October 2021 within the ripretinib EAP in our Institution was conducted. Clinician-documented and mRECIST 1.

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Background: Epithelioid hemangioendothelioma (EHE) is an ultra-rare, vascular sarcoma with clinical presentation ranging from an indolent to an aggressive form. Over 50% of patients present with metastatic disease, requiring systemic therapy, although no systemic therapies are specifically approved for EHE. Retrospective evidence supports the activity of mTOR inhibitors (e.

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