婴儿中耳胚胎型横纹肌肉瘤1例并文献复习  

A case of an infant with embryonal rhabdomyosarcoma in the middle ear and literature review

作　　者：李玲[1,2] 李聪[1,2] 孙岩 陈良[2] LI Ling;LI Cong;SUN Yan;CHEN Liang(The second Medical College of BinZhou Medical University,Yantai 264003,Shandong,China;Department of Otorhinolaryngology&Head and Neck Surgery,Yantai Yuhuangding Hospital Affiliated to Qingdao University/Shandong Provincial Clinical Research Center for Otorhinolaryngologic Diseases,Yantai 264000,Shandong,China)

机构地区：[1]滨州医学院第二临床医学院,山东烟台264003 [2]青岛大学附属烟台毓璜顶医院耳鼻咽喉头颈外科/山东省耳鼻喉疾病临床医学研究中心,山东烟台264000

出　　处：《山东大学耳鼻喉眼学报》2022年第1期55-59,共5页Journal of Otolaryngology and Ophthalmology of Shandong University

基　　金：烟台市科技计划项目任务书(2020MSGY078)。

摘　　要：目的 提高耳鼻喉科临床医师对婴儿中耳横纹肌肉瘤的认识。方法 对1例婴儿中耳胚胎型横纹肌肉瘤进行临床资料分析并复习相关文献。结果 患儿女,10月龄,患儿家长发现患儿左侧耳道肿物,伴耳道少量出血。CT及MRI提示左侧外耳道、鼓室及乳突内占位性病变,咽旁间隙、颈部、双侧腋窝淋巴结转移。通过活检手术后病理及免疫组化检查确诊为横纹肌肉瘤。患儿放化疗结束4个月后,因颅脑转移死亡。结论 发生在婴儿中耳的横纹肌肉瘤少见,且位置隐匿,早期无典型临床表现,容易造成误诊及漏诊;该病恶性程度高,临床上应争取早发现、早诊断及早治疗,提高患者生存率。Objective Raising awareness of otolaryngologists on embryonal rhabdomyosarcoma disease. Methods The clinical data of an infant with embryonal rhabdomyosarcoma in the middle ear were analyzed, and related literatures were reviewed. Results The patient was a 10-month-old female with a bleeding mass in the left external auditory canal. Computed tomography and magnetic resonance imaging revealed soft tissue density shadows in the left external auditory meatus, tympanum, and mastoid sinus that were accompanied with an enlarged lymph node in the parapharyngeal, neck and bilateral axilla. Moreover, the diagnosis of RMS was confirmed by postoperative pathological and immunohistochemical examination after biopsy. The patient died of brain metastasis four months after the end of radiation and chemotherapy. Conclusion RMS in the middle ear is rare. Due to its obscure location and lack of typical clinical manifestations in the early stage, RMS is difficult to detect and prone to misdiagnosis. The malignant degree of this disease is high. Thus, the early detection, diagnosis, and treatment of this disease should be further studied to improve the survival rate of patients.

关 键 词：中耳 胚胎型 横纹肌肉瘤 婴儿 恶性肿瘤 

分 类 号：R764.2[医药卫生—耳鼻咽喉科]