中国口腔颌面外科杂志 ›› 2022, Vol. 20 ›› Issue (3): 292-297.doi: 10.19438/j.cjoms.2022.03.016

• 临床总结 • 上一篇    下一篇

33例头颈部胚胎型横纹肌肉瘤临床与病理特点分析

徐卫靖*, 高振杰*, 付坤, 高宁, 程龙, 何巍   

  1. 郑州大学第一附属医院 口腔颌面外科,河南 郑州 450000
  • 收稿日期:2021-10-19 修回日期:2022-01-04 出版日期:2022-05-20 发布日期:2022-05-20
  • 通讯作者: 何巍,E-mail:heweizdyfy@163.com
  • 作者简介:徐卫靖(1996-),女,在读硕士研究生,E-mail: xvweijing233@126.com;高振杰(1986-),男,博士,E-mail: 38.547286@qq.com。*并列第一作者
  • 基金资助:
    河南省卫健委联合共建项目(LHGJ20190189)

Clinical, pathological analysis of 33 cases of embryonal rhabdomyosarcoma of head, neck

XU Wei-jing, GAO Zhen-jie, FU Kun, GAO Ning, CHENG Long, HE Wei   

  1. Department of Oral and Maxillofacial Surgery, The First Affiliated Hospital of Zhengzhou University. Zhengzhou 450000, Henan Province, China
  • Received:2021-10-19 Revised:2022-01-04 Online:2022-05-20 Published:2022-05-20

摘要: 目的: 总结头颈部胚胎型横纹肌肉瘤(embryonal rhabdomyosarcoma,ERMS)的临床表现、影像学特征、治疗方法及病理特点,为临床提供参考。方法: 回顾性分析2012年2月—2021年9月郑州大学第一附属医院经病理确诊为ERMS的患者33例,分析其临床病理特点。采用SPSS 19.0软件包对数据进行统计学分析。结果: 患者年龄9个月~59岁,平均年龄为(12.1±13.9)岁。就诊的主要症状为肿胀、疼痛和麻木。CT表现为低密度影,MRI在T1WI呈中等信号,T2WI呈中至高信号。免疫组织化学染色结果显示,22例Desmin阳性、23例myogenin阳性、22例Myo D1阳性、Ki-67增殖指数为57%±19%。发生颈淋巴结转移的患者,Ki-67指数显著高于无转移组[(63.5±10.96)%∶(53.4±21.5)%,P=0.022]。利用ROC曲线分析Ki-67指数对预测ERMS颈淋巴结转移的价值,颈淋巴结转移者,ROC曲线下面积为0.75(P=0.024)。Ki-67指数>55%时,敏感度为100%,特异度为65%。结论: 头颈部ERMS好发于儿童及青少年,CT、MRI结合PET-CT等检查可以指导诊断、治疗、监测复发及转移。组织病理检查是ERMS诊断的金标准,Ki-67指数与颈淋巴结转移相关。

关键词: 胚胎型横纹肌肉瘤, 头颈部, 临床特点, 病理学特点

Abstract: PURPOSE: To summarize the clinical manifestations, imaging characteristics, treatment and pathological types of embryonal rhabdomyosarcoma (ERMS) in the head and neck, in order to provide reference for the diagnosis and treatment of the disease. METHODS: Thirty-three cases with ERMS diagnosed by pathology in the First Affiliated Hospital of Zhengzhou University from February 2012 to September 2021 were analyzed retrospectively, and their clinicopathological data were analyzed. SPSS 19.0 software package was used for statistical analysis. RESULTS: The age of the patients ranged from 9 months to 59 years old, with an average age of(12.1±13.9) years. The main symptoms were swelling, pain and numbness. It showed low density on CT, medium signal on MRI on T1WI and medium to high signal on T2WI. Immunohistochemical results showed that 22 cases were Desmin positive, 23 were myogenin positive, 22 were MyoD1 positive, and Ki-67 proliferation index was 57%±19%. The Ki-67 index in cases with cervical lymph node metastasis was significantly higher than that in cases without cervical lymph node metastasis [(63.5±10.96)%:(53.4±21.5)%, P=0.022]. ROC curve was used to analyze the power of Ki-67 index in predicting cervical lymph node spread. The area under ROC curve of end-stage patients was 0.75 (P=0.024). A cut-off value of >55% resulted in sensitivity of 100% and specificity of 65%. CONCLUSIONS: Head and neck ERMS is common in children and adolescents. CT, MRI combined with PET-CT and other imaging examinations can guide the diagnosis, treatment and monitoring of recurrence and metastasis. Pathological examination is the gold standard for ERMS diagnosis. Cervical lymph node metastasis is associated with Ki-67 values.

Key words: Embryonal rhabdomyosarcoma, Head and neck, Clinical features, Pathological features

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