Cancer
via Europe PMC
Risk factors and outcome of children and young adults with initially unresected nonrhabdomyosarcoma soft tissue sarcoma: An analysis from the International Soft Tissue Sarcoma Consortium.
Weiss AR, Anderson J, Chen S, +7 more, Ferrari A
AI summary — for doctors, generated from the abstract only
Chemotherapy combined with radiotherapy gave better outcomes than no radiotherapy for children and young adults with initially unresected non-rhabdomyosarcoma soft tissue sarcoma.
What was studied
Pooled analysis of 661 patients from the INSTRuCT consortium trials (COG, EpSSG, MMT, CWS) whose initial surgery was biopsy only or R2 resection, all receiving preoperative chemotherapy.
Key findings
- 5-year event-free survival was 57.6% (95% CI 53.5-61.4)
- Histology was independently prognostic, with synovial sarcoma most favorable and MPNST least
- Large synovial sarcoma outcomes were better in newer trials than older protocols
- Chemotherapy plus pre- or postoperative RT outperformed no RT
Clinical relevance
Supports multimodal therapy including radiotherapy in this group and informs risk stratification by histology.
Who should care
Limitations
Pooled non-randomized protocols from different eras with variable RT use, so confounding by protocol is likely; the abstract is partly cut off.
Consider radiotherapy with chemotherapy for initially unresected NRSTS and refer large synovial sarcoma to contemporary trial-based care.
This is an AI-generated summary of the published abstract, not a clinical recommendation. It may not reflect the full text or subsequent updates — verify against the original source before acting on it.
Abstract
BACKGROUND
The role of neoadjuvant therapy in children, adolescents, and young adults (CAYA) with initially unresectable nonrhabdomyosarcoma soft tissue sarcoma (NRSTS) remains controversial, with recommendations varying by cooperative group. The International Soft Tissue Sarcoma Consortium (INSTRuCT) is a collaboration among North American and European study groups (Children's Oncology Group [COG], Cooperative Weichteilsarkom Studiengruppe [CWS], and European Paediatric Soft Tissue Sarcoma Study Group [EpSSG]). The prognostic value of tumor characteristics and outcome for CAYA with initially unresected localized NRSTS was analyzed.
METHODS
The INSTRuCT database was searched for all CAYA whose initial surgical intervention was biopsy only or surgery resulting in gross residual disease (R2). Study data from the COG (ARST0332), EpSSG (NRSTS-2005), International Society of Paediatric Oncology Malignant Mesenchymal Tumour Committee (MMT-95), and CWS (CWS-91, -96, and -2002P) were included. All patients received preoperative chemotherapy, which was often alkylator and/or anthracycline based. Neoadjuvant radiation (RT) was protocol specified for all ARST0332 subjects and for higher risk NRSTS-2005 subjects but not for subjects in the CWS or MMT-95 studies, where the use and timing of RT were more variable.
RESULTS
Outcomes were analyzed for 661 patients aged 5 cm (n = 484; 73%), located in the extremity (n = 354; 55%), T2 (n = 360; 55%), and N0 (n = 572; 87%). A greater percentage of patients with tumors of ≤5 cm were enrolled in European versus North American studies. Five-year event-free survival (EFS) for the entire cohort was 57.6% (95% CI, 53.5%-61.4%). EFS-independent prognostic factors were histology (synovial sarcoma most favorable and malignant peripheral nerve sheath tumor least favorable; p 5-cm) synovial sarcoma treated in the recent ARST0332 and NRSTS-2005 studies was more favorable when compared to earlier CWS and MMT-95 protocols.
CONCLUSIONS
Outcomes were more favorable with the combination of chemotherapy and RT (pre- or postoperative) versus no RT for initially unresected NRSTS. Patients with large synovial sarcoma may benefit from contemporary trial designs incorporating more of a risk-based directed neoadjuvant chemotherapy and RT approach. Higher R0 margin status rates were achieved in protocols with specified preoperative RT, which resulted in improved outcomes.
DOI
10.1002/cncr.70647
PMID
42911104
Research metadata (titles, abstracts, authors, publication dates) is sourced from Europe PMC, which aggregates content from PubMed/MEDLINE, PubMed Central, and other providers under the U.S. National Library of Medicine (NLM/NCBI). NLM does not claim copyright on abstracts; the original journal publishers or authors may hold rights to the full articles. AI-generated summaries are derived only from the abstract, never the full text, and are always labeled as AI-generated.
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