194 results match your criteria: "Nonrhabdomyosarcoma Soft Tissue Sarcomas"

Article Synopsis
  • The study investigated the expression of VEGFRs, PDGFRs, and c-Kit in pediatric patients with high-grade non-rhabdomyosarcoma soft tissue sarcoma (NRSTS) to understand their impact on growth, invasion, and treatment outcomes.
  • Out of 113 pediatric patients analyzed, about one-third showed high expression of PDGFRα, while other markers had lower expression rates; high PDGFRα and VEGFR2 were notably associated with poorer survival rates.
  • The results suggest that these tyrosine kinase receptors are upregulated in NRSTS, indicating a potential for targeted therapies to improve outcomes in affected patients.
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Objective: The current treatment of pediatric non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) is a multimodal risk-based approach. Today, smaller fields and lower doses of radiotherapy (RT) have become standard. In this study, it was aimed to evaluate the treatment outcomes and toxicity profile in children with NRSTS that received RT as a part of multimodal therapy.

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Contemporary surgical management of pediatric non-rhabdomyosarcoma soft tissue sarcoma.

Pediatr Blood Cancer

November 2024

Division of Pediatric General and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, Ohio, USA.

Non-rhabdomyosarcoma soft tissue sarcoma (STS) comprises most STS in pediatric patients. It is a diverse set of over 30 histologic subtypes. Treatment is based on risk group determined by tumor size, grade, and the presence of metastases.

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Article Synopsis
  • The study focuses on outcomes for pediatric low-grade non-rhabdomyosarcoma soft tissue sarcoma (NRSTS), analyzing data from patients under 30 years old enrolled in a specific clinical trial.
  • Low-risk patients were treated with surgery alone, leading to high survival rates (90% event-free and 100% overall), while intermediate- and high-risk groups had significantly lower survival rates (55% event-free and 25% overall) despite aggressive treatment.
  • Findings suggest that most low-risk patients can be effectively managed with surgery only, and the current grading system may over-treat some patients who do not need additional therapies beyond surgery.
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Proton Therapy in Non-Rhabdomyosarcoma Soft Tissue Sarcomas of Children and Adolescents.

Cancers (Basel)

April 2024

Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, 20133 Milano, Italy.

This paper provides insights into the use of Proton Beam Therapy (PBT) in pediatric patients with non-rhabdomyosarcoma soft tissue sarcomas (NRSTS). NRSTS are a heterogeneous group of rare and aggressive mesenchymal extraskeletal tumors, presenting complex and challenging clinical management scenarios. The overall survival rate for patients with NRSTS is around 70%, but the outcome is strictly related to the presence of various variables, such as the histological subtype, grade of malignancy and tumor stage at diagnosis.

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Background: The aim of this study was to estimate the event-free survival (EFS) of children and young adults with relapsed or refractory nonrhabdomyosarcoma soft tissue sarcoma (NRSTS) treated in nonrandomized phase 2 studies conducted by the Children's Oncology Group (COG) and predecessor groups to establish a benchmark EFS for future phase 2 NRSTS trials evaluating the activity of novel agents.

Methods: A retrospective analysis of patients with recurrent or refractory NRSTS prospectively enrolled in nonrandomized phase 2 COG and predecessor group trials between 1994 and 2015 was conducted. EFS was defined as disease progression/relapse or death and calculated via the Kaplan-Meier method.

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Article Synopsis
  • Soft tissue sarcomas make up 6%-8% of cancers in children, with rhabdomyosarcoma being the most common type at 3% of pediatric cancers, known for its high-grade nature and tendency to spread.
  • Treatment for rhabdomyosarcomas involves a risk-adapted, multimodal approach that includes surgery, radiotherapy, and chemotherapy, while other soft tissue sarcomas, which account for 3%-4% of cases, vary in grade and often require similar treatments.
  • The article focuses on staging, risk assessment, and imaging related to soft tissue sarcomas, mainly within the context of the Children's Oncology Group trials, while also integrating insights from international research collaborations.
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The human leukocyte antigen (HLA) system is a major factor controlling cancer immunosurveillance and response to immunotherapy, yet its status in pediatric cancers remains fragmentary. We determined high-confidence HLA genotypes in 576 children, adolescents and young adults with recurrent/refractory solid tumors from the MOSCATO-01 and MAPPYACTS trials, using normal and tumor whole exome and RNA sequencing data and benchmarked algorithms. There was no evidence for narrowed HLA allelic diversity but discordant homozygosity and allele frequencies across tumor types and subtypes, such as in embryonal and alveolar rhabdomyosarcoma, neuroblastoma and 11q subtypes, and high-grade glioma, and several alleles may represent protective or susceptibility factors to specific pediatric solid cancers.

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Article Synopsis
  • Pediatric non-rhabdomyosarcoma soft-tissue sarcomas (NRSTS) are tough tumors that don’t have the best treatment options for some patients, especially if they can’t be surgically removed at first.
  • A study looked at 71 patients under 21 who were treated for NRSTS from 1990 to 2021, finding that many had large, dangerous tumors and most received chemotherapy before surgery.
  • Results showed that while the survival rates were not great, patients who responded well to chemotherapy and had successful surgeries had a better chance of living longer, highlighting the need for better treatment plans.
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Soft tissue sarcomas (STS) represent a heterogeneous group of extraskeletal mesenchymal tumors that affect individuals throughout the entire age continuum. Despite this pervasive influence, key differences exist in the presentation of these sarcomas across varying age groups that have prevented a more uniform approach to management. Notably, rhabdomyosarcoma (RMS) is more common in children, while most nonrhabdomyosarcoma soft tissue sarcoma (NRSTS) subtypes are more prevalent in adults.

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Background: The aim of this study was to assess the clinical impact of indeterminate pulmonary nodules (no more than four pulmonary nodules of less than 5 mm or one nodule measuring between 5 and less than 10 mm by computed tomography [CT]) in children and adolescents with adult-type non-rhabdomyosarcoma soft tissue sarcoma (NRSTS) at diagnosis.

Methods: Patients with NRSTS treated in 11 centers as part of the European paediatric Soft Tissue Sarcoma Study Group (EpSSG) were retrospectively assessed. Local radiologists, blinded to clinical information except for patients' age and tumor histotype, reviewed the chest CT at diagnosis and filled out a case report form.

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Clinical features and outcomes of infantile soft-tissue sarcoma: A multicenter retrospective study in Beijing.

J Cancer Res Ther

August 2023

Department of Medical Oncology, Pediatric Oncology Center, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing Key Laboratory of Pediatric Hematology Oncology, Key Laboratory of Major Diseases in Children, Ministry of Education, Beijing, China.

Background: Soft-tissue sarcomas during infancy are rare and understudied. With no data on this specific condition, we performed a retrospective study of infant-onset sarcomas based on a multi-institutional cohort in Beijing, China, collected over the past decade. We reviewed infantile soft-tissue sarcomas' clinical characteristics, treatments, and outcomes.

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Phosphatase of regenerating liver 3 (PRL3) is a specific tumor antigen overexpressed in a broad range of adult cancer types. However, its physiological expression in pediatric embryonal and mesenchymal tumors and its association with clinical outcomes in children is unknown. We sought to profile the expression of PRL3 in pediatric tumors in relation to survival outcomes, expression of angiogenesis markers, and G-protein-coupled receptor (GPCR)-mitogen-activated protein kinase (MAPK) signaling targets.

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JCO ARST1321 was a phase II study designed to compare the near complete pathologic response rate after preoperative chemoradiation with/without pazopanib in children and adults with intermediate-/high-risk chemotherapy-sensitive body wall/extremity non-Rhabdomyosarcoma Soft Tissue Sarcoma (ClinicalTrials.gov identifier: NCT02180867). Enrollment was stopped early following a predetermined interim analysis that found the rate of near complete pathologic response to be significantly greater with the addition of pazopanib.

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Children's Oncology Group's 2023 blueprint for research: Soft tissue sarcomas.

Pediatr Blood Cancer

September 2023

Division of Hematology/Oncology, Department of Pediatrics, Texas Children's Cancer Center, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas, USA.

In the United States, approximately 850-900 children and adolescents each year are diagnosed with soft tissue sarcomas (STS). STS are divided into rhabdomyosarcoma (RMS) and non-rhabdomyosarcoma STS (NRSTS). RMS and NRSTS are risk stratified into low-, intermediate-, and high-risk categories, with 5-year survival rates of approximately 90%, 50%-70%, and 20%, respectively.

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Synovial sarcoma (SS) is one of the commonest non-rhabdomyosarcoma soft tissue sarcoma with limited treatment options in the relapsed and advanced settings. The combination of gemcitabine and docetaxel has demonstrated its role predominantly in leiomyosarcoma and pleomorphic sarcomas but has not been prospectively studied in SS. This trial assesses the efficacy, tolerability and quality of life (QoL) with this regimen in metastatic/unresectable locally advanced relapsed SS.

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Rationale: The infantile fibrosarcoma (IFS) is a non-rhabdomyosarcoma soft tissue sarcoma with locally aggressive properties. State of the art therapy consists of neoadjuvant chemotherapy followed by wide resection according to the criteria of the musculoskeletal tumor society.

Diagnoses: An ETV6-NTRK3 positive IFS of the distal tibia in a 21-months old child showed good response to chemotherapy.

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Background: For children with non-rhabdomyosarcoma soft tissue sarcomas, a risk-adapted treatment approach is generally used in order to minimize treatment-related morbidity and mortality in low-risk patients and maximize the benefit in high-risk patients. Our aim in this review is to discuss the prognostic factors, riskadapted treatment options and the details of radiotherapy.

Methods: The publications reached by searching the keywords `pediatric soft tissue sarcoma`, `nonrhabdomyosarcoma soft tissue sarcoma (NRSTS)`, and `radiotherapy` in Pubmed database were evaluated in detail.

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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.

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Aim: To illustrate clinicopathological features of orbital non-rhabdomyosarcoma soft tissue sarcoma (NRSTS), and to compare the treatment outcome between postoperative radiotherapy (RT) and chemotherapy in a retrospective analysis nearly 20y.

Methods: A retrospective cohort study of 56 patients with orbital NRSTS were reviewed, 34 of whom received postoperative RT, and 22 received postoperative chemotherapy. The clinicopathological features, local recurrence, metastases, and survival data were recorded.

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Trimodality Treatment of Extremity Soft Tissue Sarcoma: Where Do We Go Now?

Curr Treat Options Oncol

April 2023

Department of Pediatric Hematology Oncology, Maine Medical Center, 100 Campus Drive, Suite 107 Scarborough, Portland, ME, USA.

Extremity soft tissue sarcoma (ESTS) constitutes the majority of patients with soft tissue sarcoma (STS). Patients with localized high-grade ESTS > 5 cm in size carry a substantial risk of developing distant metastasis on follow-up. A neoadjuvant chemoradiotherapy approach can enhance local control by facilitating resection of the large and deep locally advanced tumors while trying to address distant spread by treating the micrometastasis for these high-risk ESTS.

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Background And Objectives: The impact upon wound healing of targeted molecular therapies, when incorporated into neoadjuvant therapy of soft tissue sarcoma, is largely unknown. Here, we describe wound complications following addition of pazopanib, a tyrosine kinase inhibitor (TKI), to neoadjuvant radiotherapy (RT) +/- chemotherapy for soft tissue sarcoma.

Methods: Wound complications were evaluated on dose-finding and randomized arms of ARST1321, a phase II/III study incorporating neoadjuvant RT, +/- pazopanib, +/- ifosfamide/doxorubicin (ID) for sarcoma therapy.

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Background: Margin status following surgery in children, adolescents, and young adults with soft tissue sarcomas is controversial and has been defined differently by various specialties, with definitions changing over time and by cooperative group. The International Soft Tissue Sarcoma Consortium (INSTRuCT) is a collaboration of the Children's Oncology Group (COG) Soft Tissue Sarcoma Committee, European pediatric Soft Tissue sarcoma Study Group (EpSSG), and the European Cooperative Weichteilsarkom Studiengruppe (CWS) devoted to improving patient outcomes by pooling and mining cooperative group clinical trial data.

Methods: The INSTRuCT non-rhabdomyosarcoma soft tissue sarcoma (NRSTS) working group aimed to develop international harmonized recommendations regarding surgical margin assessment and definitions in children and adolescents with soft tissue tumors.

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Abdominal Wall Extraskeletal Ewing's Sarcoma in an 8-Year-Old Child.

J Indian Assoc Pediatr Surg

November 2022

Department of Pediatric Surgery, Indira Gandhi Institute of Child Health, Bengaluru, Karnataka, India.

Nonrhabdomyosarcoma soft-tissue sarcomas (NRSTSs) are a heterogeneous group of neoplasms of presumed mesenchymal origin. The precise diagnosis of the type of NRSTS tumor is complex. Among them, Ewing's sarcoma is very rare.

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Purpose: The aim of this paper is to better define the clinical features and outcomes of young patients with non-rhabdomyosarcoma soft tissue sarcoma (NRSTS) with regional and distant lymph node (LN) metastases treated in a standardised fashion, we analysed LN involvement in COG study ARST0332, which evaluated a risk-based treatment strategy for young patients with all stages of NRSTS.

Patients And Methods: Patients <30 years old with newly diagnosed NRSTS and LN metastases enrolled on ARST0332 were studied. Regional LN sampling was required for those with epithelioid sarcoma, clear cell sarcoma or clinically/radiographically enlarged LNs.

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