罕见新生儿鼻咽部横纹肌肉瘤1例及文献复习

Rare nasopharyngeal rhabdomyosarcoma in the newborn: a cases report and literature review

摘要:

目的 通过报道1例新生儿鼻咽部横纹肌肉瘤（RMS）及其相关文献复习，以提高临床医师对该病的认识。方法 患者，女，16 d，出生后即发现有鼻塞、张口呼吸，吸吮时憋气、呼吸困难。鼻咽喉内镜、CT及MRI检查提示鼻咽部占位性病变，通过活检手术后病理及免疫组化检查确诊为RMS。结果 患儿经系统化疗后病情好转，8个月龄时内镜及影像学复查，局部病灶较前缩小，一般状况良好，未见头颈及全身转移。结论 发生在新生儿鼻咽部的RMS十分罕见，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。

Abstract:

Objective This paper reports a case of neonatal nasopharyngeal rhabdomyosarcoma (RMS) and reviews the related literature in order to improve the clinician awareness of the disease. Methods The patient, female, 16 days old, was found to have nasal obstruction and open-mouth breathing after birth, and suffocated and dyspnea when sucking. The examinations of Nasopharyngeal endoscopy, CT and MRI suggested space-occupying lesions in the nasopharynx. And the diagnosis of RMS was confirmed by postoperative pathological and immunohistochemical examination after biopsy. Results The patient's condition was improved after systemic chemotherapy. The endoscopy and imaging reexamination at the age of 8 months showed that the local lesions were smaller than before, and the general condition was good. No head, neck or systemic metastasis of the patient was observed. Conclusion RMS in neonatal nasopharynx is very rare, with hidden location and no typical clinical manifestations in early stage, which is easy to cause misdiagnosis and missed diagnosis. The malignant degree of this disease is high. Early detection, early diagnosis and early treatment of this disease should be tried to improve the survival rate of patients.