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成人头颈部横纹肌肉瘤:基于放疗的治疗、结果和生存预测因素。

Adult head and neck rhabdomyosarcoma: radiotherapy- based treatment, outcomes, and predictors of survival.

机构信息

Key Laboratory of Carcinogenesis and Translational Research (Ministry of Education/Beijing), Department of Radiation Oncology, Peking University Cancer Hospital & Institute, Peking University, 100142, Beijing, P.R. China.

Department of Radiotherapy, The Affiliated Yantai Yuhuangding Hospital of Qingdao University, Shandong, P.R. China.

出版信息

BMC Cancer. 2024 Mar 14;24(1):340. doi: 10.1186/s12885-024-12079-y.

Abstract

BACKGROUND

Adult head and neck rhabdomyosarcoma (HNRMS) is an exceptionally rare malignancy, and there is a paucity of data and research dedicated to understanding its characteristics and management in adult populations. This study aimed to assess the outcomes and identify survival predictors in adult HNRMS.

METHODS

We retrospectively evaluated 42 adult patients (> 16 years) with HNRMS who received radiotherapy (RT)-based treatment at our institute between 2008 and 2022. We analysed the clinical characteristics and prognosis of these patients, including the locoregional recurrence-free survival (LRFS), progression-free survival (PFS), and overall survival (OS), using the Kaplan-Meier method. The chi-square and Fisher's exact tests were used to analyse differences between groups for dichotomous and categorical variables, respectively. Survival rates were calculated using the Kaplan-Meier method. Prognostic variables were assessed through univariate Cox analyses.

RESULTS

The median patient age was 28 years (range, 16-82 years). Alveolar RMS was the most common histological type, observed in 21 patients (50.0%), followed by embryonal in 16 patients (38.1%). The anatomic sites of origin were orbital in one (2.4%), parameningeal in 26 (61.9%), and non-orbital/non-parameningeal in 15 (35.7%) patients. Nineteen patients (45.2%) had regional lymph node metastasis, and five patients (11.9%) presented with distant metastatic disease. Distant metastasis (n = 17) was the primary cause of treatment failure. At a median follow-up of 47.0 months, the 5-year LRFS, PFS, and OS rates were 69.0%, 39.7%, and 41.0%, respectively. Univariate analysis revealed that tumour size, lymph node involvement, and the local treatment pattern (surgery and RT vs. RT alone) were significant predictors of survival.

CONCLUSIONS

The main failure pattern in patients with HNRMS receiving RT-based treatment was distant metastasis. Tumour size > 5 cm and lymph node involvement were predictors of worse LRFS. Multimodality local treatment, combining surgery and RT, is effective and provides survival benefits.

摘要

背景

成人头颈部横纹肌肉瘤(HNRMS)是一种极为罕见的恶性肿瘤,针对成人人群中该疾病的特征和管理,相关数据和研究都十分有限。本研究旨在评估成人 HNRMS 的治疗结果并确定其生存预测因素。

方法

我们回顾性评估了 2008 年至 2022 年期间在我们研究所接受基于放疗(RT)治疗的 42 例 HNRMS 成人患者(>16 岁)。我们使用 Kaplan-Meier 方法分析了这些患者的临床特征和预后,包括局部区域无复发生存(LRFS)、无进展生存(PFS)和总体生存(OS)。使用卡方检验和 Fisher 确切概率法分别对组间二分类变量和分类变量进行分析。使用 Kaplan-Meier 法计算生存率。通过单因素 Cox 分析评估预后变量。

结果

患者的中位年龄为 28 岁(范围 16-82 岁)。最常见的组织学类型为腺泡型横纹肌肉瘤,共 21 例(50.0%),其次是胚胎型横纹肌肉瘤,共 16 例(38.1%)。起源解剖部位为眶内 1 例(2.4%)、颅底 26 例(61.9%)和非眶内/非颅底 15 例(35.7%)。19 例(45.2%)患者有区域淋巴结转移,5 例(11.9%)患者有远处转移。远处转移(n=17)是治疗失败的主要原因。中位随访 47.0 个月时,5 年 LRFS、PFS 和 OS 率分别为 69.0%、39.7%和 41.0%。单因素分析显示,肿瘤大小、淋巴结受累和局部治疗方式(手术联合 RT 与单纯 RT)是生存的显著预测因素。

结论

接受基于 RT 治疗的 HNRMS 患者的主要失败模式是远处转移。肿瘤大小>5cm 和淋巴结受累是 LRFS 更差的预测因素。手术联合 RT 的多模态局部治疗是有效的,可带来生存获益。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/be47/10938762/877cf4f527fb/12885_2024_12079_Fig1_HTML.jpg

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