{"doi":"10.1002/pbc.31062","title":"Clinical features and outcomes of young patients with low‐grade non‐rhabdomyosarcoma soft tissue sarcomas treated with a risk‐based strategy: A report from Children's Oncology Group study ARST0332","abstract":"BACKGROUND: In retrospective analyses, the Pediatric Oncology Group [POG) and the Federation National des Centres de Lutte Contre le Cancer (FNCLCC) histologic grade predict outcome in pediatric non-rhabdomyosarcoma soft tissue sarcoma (NRSTS), but prospective data on grading, clinical features, and outcomes of low-grade NRSTS are limited. METHODS: We analyzed patients less than 30 years of age enrolled on Children's Oncology Group (COG) study ARST0332 (NCT00346164) with POG grade 1 or 2 NRSTS. Low-risk patients were treated with surgery alone. Intermediate-/high-risk patients received ifosfamide/doxorubicin and radiotherapy, with definitive resection either before or after 12 weeks of chemoradiotherapy. RESULTS: Estimated 5-year event-free and overall survival were 90% and 100% low risk (n = 80), 55% and 78% intermediate risk (n = 15), and 25% and 25% high risk (n = 4). In low-risk patients, only local recurrence was seen in 10%; none with margins greater than 1 mm recurred locally. Sixteen of 17 intermediate-/high-risk patients who completed neoadjuvant chemoradiotherapy underwent gross total tumor resection, 80% with negative margins. Intermediate-/high-risk group events included one local and seven metastatic recurrences. Had the FNCLCC grading system been used to direct treatment, 29% of low-risk (surgery alone) patients would have received radiotherapy ± chemotherapy. CONCLUSIONS: Most low-risk patients with completely resected POG low-grade NRSTS are successfully treated with surgery alone, and surgical margins greater than 1 mm may be sufficient to prevent local recurrence. Patients with intermediate- and high-risk low-grade NRSTS have outcomes similar to patients with high-grade histology, and require more effective therapies. Use of the current FNCLCC grading system may result in overtreatment of low-risk NRSTS curable with surgery alone.","journal":"Pediatric Blood & Cancer","year":2024,"id":460964,"datarank":0.0,"base_score":0.0,"endowment":0.0,"self_citation_contribution":0.0,"citation_network_contribution":0.0,"self_endowment_contribution":0.0,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":3,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":0.9508,"is_data_producer":false,"deposit_databanks":null,"is_oa":true,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":"2024-01-01","fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":1289523,"name":"Cheryl M. Coffin","orcid":null,"position":1,"is_corresponding":false},{"id":411468,"name":"R. Lor Randall","orcid":"0000-0002-3660-774X","position":2,"is_corresponding":false},{"id":1289081,"name":"Yan Yang","orcid":"0000-0002-7851-9246","position":3,"is_corresponding":false},{"id":277181,"name":"Donald A. Barkauskas","orcid":"0000-0002-2339-719X","position":4,"is_corresponding":false},{"id":492634,"name":"Lynn Million","orcid":"0000-0002-5931-4915","position":5,"is_corresponding":false},{"id":384389,"name":"M. Beth McCarville","orcid":null,"position":6,"is_corresponding":false},{"id":267020,"name":"Alberto S. Pappo","orcid":"0000-0002-5338-3979","position":7,"is_corresponding":false},{"id":258741,"name":"Aaron R. Weiss","orcid":"0000-0001-8352-3963","position":8,"is_corresponding":false},{"id":383475,"name":"Sheri L. Spunt","orcid":"0000-0002-1669-2676","position":9,"is_corresponding":false},{"id":1289080,"name":"David P. Douglass","orcid":"0000-0001-5245-0917","position":0,"is_corresponding":true}],"reference_count":26,"raw_metadata":null,"created_at":"2026-07-19T02:04:07.925246Z","pmid":"38757485","pmcid":null,"fwci":null,"citation_percentile":null,"influential_citations":0,"oa_status":null,"license":null,"views":0,"total_file_size_bytes":0,"version_count":0,"fair_f":null,"fair_a":null,"fair_i":null,"fair_r":null,"fair_zscore":null,"fair_rationale":null,"fair_model":null,"fair_agent_version":null,"fair_fulltext_source":null,"fair_has_llm":null,"fair_computed_at":null,"clinical_trials":[],"software_tools":[],"db_accessions":[],"linked_datasets":[],"topics":[]}