Localized incompletely resected standard risk rhabdomyosarcoma in children and adolescents: Results from the European Paediatric Soft Tissue Sarcoma Study Group RMS 2005 trial.

IF 6.1 2区医学 Q1 ONCOLOGY

Cancer Pub Date : 2024-12-01 Epub Date: 2024-07-26 DOI:10.1002/cncr.35497

Henry C Mandeville, Gianni Bisogno, Veronique Minard-Colin, Rita Alaggio, Myriam Ben-Arush, Cyrus Chargari, Beatrice Coppadoro, Ross Craigie, Christine Devalck, Sima Ferman, Andrea Ferrari, Heidi Glosli, Raquel Hladun Alvaro, Marinka Hol, Peter Mudry, Daniel Orbach, Monica Ramos Albiac, Johannes H M Merks, Meriel E M Jenney

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引用次数: 0

Abstract

Background: The authors report the prospective evaluation of reduced dose alkylator chemotherapy combined with radiotherapy for European Pediatric Soft Tissue Sarcoma Study Group (EpSSG) standard risk nonalveolar rhabdomyosarcoma (NA-RMS).

Patients and methods: Localized node negative Intergroup Rhabdomyosarcoma Study (IRS) II/III NA-RMS at favorable sites (subgroup C), <25 years old, received five cycles of ifosfamide, vincristine, and dactinomycin (IVA) chemotherapy (30 g/m² ifosfamide) and four cycles of vincristine and dactinomycin (if receiving radiotherapy), or nine cycles of IVA (54 g/m² ifosfamide) ± radiotherapy. Delayed primary tumor excision was considered for IRS III tumors. The primary end points were event-free survival (EFS) and overall survival (OS).

Results: From October 2005 to December 2016, 359 evaluable patients were recruited: orbit, 164 (45.7%); head and neck nonparameningeal, 77 (21.4%); and genitourinary non-bladder/prostate, 118 (32.9%). EFS and OS were 77.4% (95% confidence interval [CI], 72.5-81.6) and 93.5% (95% CI, 90.1-95.8), respectively. Lower dose alkylator chemotherapy and radiotherapy achieved 5-year OS of 93.7% but the difference with higher dose alkylator chemotherapy +/- radiotherapy was not significant (p = 0.8003). Adjuvant radiotherapy improved EFS with 5-year estimates of 84.7% versus 65.2% for nonirradiated (p < .0001), but not OS (p = .9298). Omitting radiotherapy for orbital tumors reduced OS (5-year was 87.1% vs. 97.3% for irradiated, p = .0257). Following R0 resection (n = 60), radiotherapy did not significantly improve EFS or OS.

Conclusions: Radiotherapy for local tumor control allows for reduction of cumulative dose of alkylators in EpSSG standard risk subgroup C RMS patients. The omission of radiotherapy did not affect OS in all patients except those with orbital RMS and was associated with inferior EFS.

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儿童和青少年局部未完全切除的标准风险横纹肌肉瘤：欧洲儿童软组织肉瘤研究小组 RMS 2005 试验结果。

研究背景作者报告了对欧洲小儿软组织肉瘤研究组（EpSSG）标准风险非肺泡横纹肌肉瘤（NA-RMS）减量烷化剂化疗联合放疗的前瞻性评估：在有利部位（C亚组）进行局部结节阴性的Intergroup Rhabdomyosarcoma Study（IRS）II/III NA-RMS，2次ifosfamide）和4个周期的长春新碱和双霉素（如果接受放疗），或9个周期的IVA（54克/平方米ifosfamide）±放疗。对于IRS III肿瘤，考虑延迟原发肿瘤切除术。主要终点为无事件生存期（EFS）和总生存期（OS）：2005年10月至2016年12月，共招募了359例可评估患者：其中眼眶肿瘤164例（45.7%）；头颈部非副脑膜肿瘤77例（21.4%）；泌尿生殖系统非膀胱/前列腺肿瘤118例（32.9%）。EFS 和 OS 分别为 77.4%（95% 置信区间 [CI]，72.5-81.6）和 93.5%（95% 置信区间 [CI]，90.1-95.8）。低剂量烷化剂化疗和放疗的5年生存率为93.7%，但与高剂量烷化剂化疗+/-放疗相比，差异不显著（P = 0.8003）。辅助放疗改善了患者的生存期，5 年生存率估计为 84.7%，而未接受放疗的患者为 65.2%（p 结论：辅助放疗改善了患者的生存期，5 年生存率估计为 84.7%，而未接受放疗的患者为 65.2%：在EpSSG标准风险C亚组RMS患者中，局部肿瘤控制放疗可减少烷化剂的累积剂量。除眼眶RMS患者外，不进行放疗不会影响所有患者的OS，但与较差的EFS有关。

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来源期刊

Cancer 医学-肿瘤学

CiteScore

13.10

自引率

3.20%

发文量

480

审稿时长

2-3 weeks

期刊介绍： The CANCER site is a full-text, electronic implementation of CANCER, an Interdisciplinary International Journal of the American Cancer Society, and CANCER CYTOPATHOLOGY, a Journal of the American Cancer Society. CANCER publishes interdisciplinary oncologic information according to, but not limited to, the following disease sites and disciplines: blood/bone marrow; breast disease; endocrine disorders; epidemiology; gastrointestinal tract; genitourinary disease; gynecologic oncology; head and neck disease; hepatobiliary tract; integrated medicine; lung disease; medical oncology; neuro-oncology; pathology radiation oncology; translational research