AI 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.

Article Abstract

Background: Pediatric non-rhabdomyosarcoma soft-tissue sarcomas (NRSTS) are a heterogeneous group of aggressive tumors. Patients with locally advanced/initially unresected disease represent a subset of patients with unsatisfactory outcome: limited data are available on the best treatment approach, in particular regarding local therapy.

Methods: This retrospective analysis concerned 71 patients < 21 years old with nonmetastatic, initially unresected adult-type NRSTS, treated at a referral center for pediatric sarcomas from 1990 to 2021. Patients were treated using a multimodal approach, based on the protocols adopted at the time of their diagnosis.

Results: The series included a selected group of patients with unfavorable clinical characteristics, i.e., most cases had high-grade and large tumors, arising from axial sites in 61% of cases. All patients received neoadjuvant chemotherapy, 58 (82%) had delayed surgery (R0 in 45 cases), and 50 (70%) had radiotherapy. Partial response to chemotherapy was observed in 46% of cases. With a median follow-up of 152 months (range, 18-233), 5-year event-free survival (EFS) and overall survival (OS) were 39.9% and 56.5%, respectively. Survival was significantly better for patients who responded to chemotherapy, and those who had a delayed R0 resection. Local relapse at 5 years was 7.7% for patients who did not undergo delayed surgery.

Conclusions: Our series underscores the unsatisfactory outcome of initially unresected NRSTS patients. Improving the outcome of this patient category requires therapeutic strategies able to combine novel effective systemic therapies with a better-defined local treatment approach to offer patients the best chances to have R0 surgery.

Download full-text PDF

Source
http://dx.doi.org/10.1002/pbc.30901DOI Listing

Publication Analysis

Top Keywords

non-rhabdomyosarcoma soft-tissue
8
soft-tissue sarcomas
8
local treatment
4
treatment initially
4
initially unresected
4
unresected non-rhabdomyosarcoma
4
sarcomas children
4
children adolescents
4
adolescents retrospective
4
retrospective single-center
4

Similar Publications

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.
View Article and Find Full Text PDF

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.

View Article and Find Full Text PDF

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.

View Article and Find Full Text PDF
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.
View Article and Find Full Text PDF

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.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!