{"doi":"10.1002/pbc.31303","title":"Outcomes for patients with perineal and perianal rhabdomyosarcoma: A report from the Children's Oncology Group Soft Tissue Sarcoma Committee","abstract":"PURPOSE: To describe clinical features, risk factors, and outcomes of patients with perineal and perianal rhabdomyosarcoma. METHODS: The records of 51 patients (38 perineal and 13 perianal) enrolled on Children's Oncology Group clinical trials between 1997 and 2012 were reviewed. RESULTS: At presentation, 53% were female, 65% were older than 10 years of age, 76% were alveolar histology, 76% were more than 5 cm, 84% were invasive, 65% were regional node positive by imaging, 49% were metastatic, only 16% were grossly resected upfront, and 25% of patients had a delayed excision. At a median follow-up of 6.13 years, estimated 5-year event-free survival (EFS) was 38% [22.17%-53.38%], and overall survival (OS) was 42% [26.66%-58.21%]. The rates of local, regional, and distant failure were 15.6%, 13.7%, 43.1%, respectively; all failures ultimately died. By univariate analysis, only age more than 10 years negatively impacted 5-year EFS (p = .023) and OS (p = .09), and IRS Group also impacted OS (p = .043). In Cox proportional hazards model, neither of these variables were significant after adjusting for other factors. CONCLUSION: Patients with perineal and perianal rhabdomyosarcoma have a poor overall prognosis, probably related to poor patient and disease characteristics at presentation.","journal":"Pediatric Blood & Cancer","year":2024,"id":471805,"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":2,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":0.7517,"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":282178,"name":"Wei Xue","orcid":"0000-0002-5566-8329","position":1,"is_corresponding":false},{"id":1293274,"name":"Andrea Hayes Dixon","orcid":null,"position":2,"is_corresponding":false},{"id":281408,"name":"Suzanne L. Wolden","orcid":"0000-0002-5234-4134","position":3,"is_corresponding":false},{"id":477587,"name":"Torunn I. Yock","orcid":"0000-0001-6408-8011","position":4,"is_corresponding":false},{"id":335001,"name":"Rajkumar Venkatramani","orcid":"0000-0002-4785-106X","position":5,"is_corresponding":false},{"id":335002,"name":"David A. Rodeberg","orcid":"0000-0003-3594-7547","position":6,"is_corresponding":false},{"id":433569,"name":"Roshni Dasgupta","orcid":"0000-0003-4839-4937","position":0,"is_corresponding":true}],"reference_count":31,"raw_metadata":null,"created_at":"2026-07-19T02:05:48.788780Z","pmid":"39228061","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":[]}