Reineke A Schoot, Pieter Taselaar, Giovanni Scarzello, Frederic Kolb, Beatrice Coppadoro, Simone Ter Horst, Henry Mandeville, Andrea Ferrari, Raquel Hladun, Sylvie Helfre, Sima Ferman, Anna Kelsey, Marinka L F Hol, Christine Devalck, Myriam Ben-Arush, Daniel Orbach, Julia Chisholm, Meriel Jenney, Veronique Minard-Colin, Gianni Bisogno, Johannes H M Merks
{"title":"副脑膜横纹肌肉瘤：欧洲儿科软组织肉瘤研究小组 RMS 2005 研究结果。","authors":"Reineke A Schoot, Pieter Taselaar, Giovanni Scarzello, Frederic Kolb, Beatrice Coppadoro, Simone Ter Horst, Henry Mandeville, Andrea Ferrari, Raquel Hladun, Sylvie Helfre, Sima Ferman, Anna Kelsey, Marinka L F Hol, Christine Devalck, Myriam Ben-Arush, Daniel Orbach, Julia Chisholm, Meriel Jenney, Veronique Minard-Colin, Gianni Bisogno, Johannes H M Merks","doi":"10.1002/hed.27994","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>Parameningeal (PM) site is an unfavorable characteristic in rhabdomyosarcoma (RMS). We described the treatment and outcome for patients with PM RMS and investigated the prognostic value of risk factors. We scored PM site by originating site and by highest risk extension.</p><p><strong>Methods: </strong>Patients with PM RMS were treated within the European pediatric Soft tissue sarcoma Study Group (EpSSG) RMS 2005 study with risk-adapted, multi-modal treatment.</p><p><strong>Results: </strong>Three-hundred-eighty-one patients with PM RMS were included. Radiotherapy was administered in 359 patients (77 with surgery). After a median follow-up of 75 months, 5-year event-free survival was 60% (95% confidence interval (CI) 55%-65%), 5-year overall survival was 65% (95% CI 60%-70%).</p><p><strong>Conclusions: </strong>The outcome for patients with PM RMS has not improved in comparison to previous historical studies, despite the more rigorous application of radiotherapy (94% of patients). Signs of meningeal involvement, PM site, and age at diagnosis remained prognostic risk factors.</p><p><strong>Trial registration: </strong>EudraCT number 2005-000217-35.</p>","PeriodicalId":55072,"journal":{"name":"Head and Neck-Journal for the Sciences and Specialties of the Head and Neck","volume":" ","pages":""},"PeriodicalIF":2.3000,"publicationDate":"2024-11-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Parameningeal Rhabdomyosarcoma: Results of the European Pediatric Soft Tissue Sarcoma Study Group RMS 2005 Study.\",\"authors\":\"Reineke A Schoot, Pieter Taselaar, Giovanni Scarzello, Frederic Kolb, Beatrice Coppadoro, Simone Ter Horst, Henry Mandeville, Andrea Ferrari, Raquel Hladun, Sylvie Helfre, Sima Ferman, Anna Kelsey, Marinka L F Hol, Christine Devalck, Myriam Ben-Arush, Daniel Orbach, Julia Chisholm, Meriel Jenney, Veronique Minard-Colin, Gianni Bisogno, Johannes H M Merks\",\"doi\":\"10.1002/hed.27994\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Background: </strong>Parameningeal (PM) site is an unfavorable characteristic in rhabdomyosarcoma (RMS). We described the treatment and outcome for patients with PM RMS and investigated the prognostic value of risk factors. We scored PM site by originating site and by highest risk extension.</p><p><strong>Methods: </strong>Patients with PM RMS were treated within the European pediatric Soft tissue sarcoma Study Group (EpSSG) RMS 2005 study with risk-adapted, multi-modal treatment.</p><p><strong>Results: </strong>Three-hundred-eighty-one patients with PM RMS were included. Radiotherapy was administered in 359 patients (77 with surgery). After a median follow-up of 75 months, 5-year event-free survival was 60% (95% confidence interval (CI) 55%-65%), 5-year overall survival was 65% (95% CI 60%-70%).</p><p><strong>Conclusions: </strong>The outcome for patients with PM RMS has not improved in comparison to previous historical studies, despite the more rigorous application of radiotherapy (94% of patients). Signs of meningeal involvement, PM site, and age at diagnosis remained prognostic risk factors.</p><p><strong>Trial registration: </strong>EudraCT number 2005-000217-35.</p>\",\"PeriodicalId\":55072,\"journal\":{\"name\":\"Head and Neck-Journal for the Sciences and Specialties of the Head and Neck\",\"volume\":\" \",\"pages\":\"\"},\"PeriodicalIF\":2.3000,\"publicationDate\":\"2024-11-15\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Head and Neck-Journal for the Sciences and Specialties of the Head and Neck\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1002/hed.27994\",\"RegionNum\":3,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q1\",\"JCRName\":\"OTORHINOLARYNGOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Head and Neck-Journal for the Sciences and Specialties of the Head and Neck","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1002/hed.27994","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"OTORHINOLARYNGOLOGY","Score":null,"Total":0}
Parameningeal Rhabdomyosarcoma: Results of the European Pediatric Soft Tissue Sarcoma Study Group RMS 2005 Study.
Background: Parameningeal (PM) site is an unfavorable characteristic in rhabdomyosarcoma (RMS). We described the treatment and outcome for patients with PM RMS and investigated the prognostic value of risk factors. We scored PM site by originating site and by highest risk extension.
Methods: Patients with PM RMS were treated within the European pediatric Soft tissue sarcoma Study Group (EpSSG) RMS 2005 study with risk-adapted, multi-modal treatment.
Results: Three-hundred-eighty-one patients with PM RMS were included. Radiotherapy was administered in 359 patients (77 with surgery). After a median follow-up of 75 months, 5-year event-free survival was 60% (95% confidence interval (CI) 55%-65%), 5-year overall survival was 65% (95% CI 60%-70%).
Conclusions: The outcome for patients with PM RMS has not improved in comparison to previous historical studies, despite the more rigorous application of radiotherapy (94% of patients). Signs of meningeal involvement, PM site, and age at diagnosis remained prognostic risk factors.
Trial registration: EudraCT number 2005-000217-35.
期刊介绍：
Head & Neck is an international multidisciplinary publication of original contributions concerning the diagnosis and management of diseases of the head and neck. This area involves the overlapping interests and expertise of several surgical and medical specialties, including general surgery, neurosurgery, otolaryngology, plastic surgery, oral surgery, dermatology, ophthalmology, pathology, radiotherapy, medical oncology, and the corresponding basic sciences.
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