Häußler Sophia Marie, Stromberger Carmen, Olze Heidi, Seifert Georg, Knopke Steffen, Böttcher Arne
Department of Otorhinolaryngology, Head and Neck Surgery, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Campus Virchow-Klinikum, Augustenbuger Platz 1, 13353, Berlin, Germany.
Department of Radiation Oncology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Campus Virchow-Klinikum, Berlin, Germany.
J Cancer Res Clin Oncol. 2018 Feb;144(2):371-379. doi: 10.1007/s00432-017-2544-x. Epub 2017 Nov 16.
The purpose of this study was to assess rhabdomyosarcomas (RMS) of the head and neck in pediatric patients with regard to clinical presentation, treatment, and survival.
Data were retrospectively obtained from patient charts with regard to RMS of the head and neck diagnosed between 1996 and 2016 at a tertiary referral center. Clinical course, treatment modalities, and side effects were analyzed. Survival analysis was carried out using the Kaplan-Meier method.
Twenty-eight patients (17 male, 11 female) with a mean age at diagnosis of 6.8 ± 5.0 years have been included. Fourteen patients (50%) presented with painless swelling in the head and neck region and nine patients (32.1%) presented with cranial nerve deficit at initial diagnosis. The location of the majority of rhabdomyosarcomas was orbital (N = 9; 32.1%), followed by parapharyngeal (n = 4; 14.2%) and sinonasal (n = 3; 10.7%). All patients (n = 28; 100%) received polychemotherapy, additional adjuvant radiation therapy was carried out in 24 patients (85.7%), and surgery was performed in 12 cases (42.9%). The 5-year overall survival was 91.3%, and median progression-free survival was 46 ± 67.4 months. Common side effects resulting from chemotherapy and radiation therapy included neutropenia, mucositis, nausea and vomiting, and radiodermatitis, long-term side effects included microsomia and facial mutilation.
Multimodality treatment of rhabdomyosarcomas of the head and neck in pediatric patients may lead to a 5-year overall survival of up to 91.3%.
本研究旨在评估小儿患者头颈部横纹肌肉瘤（RMS）的临床表现、治疗及生存情况。
回顾性收集1996年至2016年在一家三级转诊中心诊断为头颈部RMS的患者病历数据。分析临床病程、治疗方式及副作用。采用Kaplan-Meier法进行生存分析。
纳入28例患者（男17例，女11例），诊断时平均年龄为6.8±5.0岁。14例患者（50%）初诊时表现为头颈部无痛性肿胀，9例患者（32.1%）初诊时出现颅神经功能缺损。大多数横纹肌肉瘤位于眼眶（n = 9；32.1%），其次是咽旁（n = 4；14.2%）和鼻窦（n = 3；10.7%）。所有患者（n = 28；100%）均接受了多药化疗，24例患者（85.7%）接受了额外的辅助放疗，12例患者（42.9%）接受了手术治疗。5年总生存率为91.3%，无进展生存期中位数为46±67.4个月。化疗和放疗的常见副作用包括中性粒细胞减少、粘膜炎、恶心和呕吐以及放射性皮炎，长期副作用包括小颌畸形和面部毁容。
小儿患者头颈部横纹肌肉瘤的多模式治疗可能使5年总生存率高达91.3%。
