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

IF 2.3 3区医学 Q1 OTORHINOLARYNGOLOGY Head and Neck-Journal for the Sciences and Specialties of the Head and Neck Pub Date : 2024-11-15 DOI:10.1002/hed.27994

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

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Abstract

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.

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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。

背景：副脑膜（PM）部位是横纹肌肉瘤（RMS）的一个不利特征。我们描述了PM RMS患者的治疗和预后，并研究了风险因素的预后价值。我们按原发部位和最高风险扩展部位对PM部位进行了评分：在欧洲儿科软组织肉瘤研究小组（EpSSG）2005年RMS研究中，对原发性RMS患者进行了风险适应性多模式治疗：结果：共纳入381名PM RMS患者。359名患者接受了放疗（77名患者接受了手术）。中位随访75个月后，5年无事件生存率为60%（95%置信区间为55%-65%），5年总生存率为65%（95%置信区间为60%-70%）：尽管放疗的应用更加严格（94%的患者），但与以往的研究相比，原发性红斑狼疮患者的预后并没有改善。脑膜受累的体征、PM部位和诊断时的年龄仍是预后风险因素：试验注册：EudraCT 编号 2005-000217-35。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Head and Neck-Journal for the Sciences and Specialties of the Head and Neck 医学-耳鼻喉科学

CiteScore

7.00

自引率

6.90%

发文量

278

审稿时长

1.6 months

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.