Parameningeal Rhabdomyosarcoma: Results of the European Pediatric Soft Tissue Sarcoma Study Group RMS 2005 Study

Other authors

Institut Català de la Salut

[Schoot RA, Taselaar P] Princess Máxima Center for Pediatric Oncology, Utrecht, The Netherlands. [Scarzello G] Veneto Institute of Oncology IOV—IRCCS, Padua, Italy. [Kolb F] Plastic and Reconstructive Surgery, UC San Diego, University of California, San Diego, California, USA. [Coppadoro B] Department of Women's and Children's Health, Hematology Oncology Division, University of Padua, Padua, Italy. [Horst ST] Princess Máxima Center for Pediatric Oncology, Utrecht, The Netherlands. Department of Radiology and Nuclear Medicine, University Medical Center Utrecht, Utrecht, The Netherlands. [Hladun R] Servei d’Hematologia i Oncologia Pediàtriques, Vall d’Hebron Hospital Universitari, Barcelona, Spain. Universitat Autònoma de Barcelona, Bellaterra, Spain

Vall d'Hebron Barcelona Hospital Campus

Publication date

2025-03-07T11:39:04Z

2025-03-07T11:39:04Z

2025-03



Abstract

Head and neck; Prognostic risk factors; Rhabdomyosarcoma


Cabeza y cuello; Factores de riesgo pronóstico; Rabdomiosarcoma


Cap i coll; Factors de risc pronòstic; Rabdomiosarcoma


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


This work was supported by Royal Marsden Cancer Charity, Alice's Arc, children's cancer charity, and NIHR Biomedical Research Centre, Royal Marsden NHS Foundation Trust/Institute of Cancer Research.

Document Type

Article


Published version

Language

English

Subjects and keywords

Infants; Tumors de parts toves - Tractament; Músculs - Càncer - Prognosi; Avaluació de resultats (Assistència sanitària); DISEASES::Neoplasms::Neoplasms by Histologic Type::Neoplasms, Connective and Soft Tissue::Neoplasms, Muscle Tissue::Myosarcoma::Rhabdomyosarcoma; Other subheadings::Other subheadings::/therapy; ANALYTICAL, DIAGNOSTIC AND THERAPEUTIC TECHNIQUES, AND EQUIPMENT::Diagnosis::Prognosis; DISEASES::Neoplasms::Neoplasms by Site::Nervous System Neoplasms::Central Nervous System Neoplasms::Meningeal Neoplasms; Other subheadings::Other subheadings::/therapy; NAMED GROUPS::Persons::Age Groups::Child; ANALYTICAL, DIAGNOSTIC AND THERAPEUTIC TECHNIQUES, AND EQUIPMENT::Diagnosis::Prognosis::Treatment Outcome; ENFERMEDADES::neoplasias::neoplasias por tipo histológico::neoplasias de tejido conjuntivo y de tejidos blandos::neoplasias de tejido muscular::miosarcoma::rabdomiosarcoma; Otros calificadores::Otros calificadores::/terapia; TÉCNICAS Y EQUIPOS ANALÍTICOS, DIAGNÓSTICOS Y TERAPÉUTICOS::diagnóstico::pronóstico; ENFERMEDADES::neoplasias::neoplasias por localización::neoplasias del sistema nervioso::neoplasias del sistema nervioso central::neoplasias meníngeas; Otros calificadores::Otros calificadores::/terapia; DENOMINACIONES DE GRUPOS::personas::Grupos de Edad::niño; TÉCNICAS Y EQUIPOS ANALÍTICOS, DIAGNÓSTICOS Y TERAPÉUTICOS::diagnóstico::pronóstico::resultado del tratamiento

Publisher

Wiley

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Rights

Attribution-NonCommercial-NoDerivatives 4.0 International

http://creativecommons.org/licenses/by-nc-nd/4.0/

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