Aggarwal Sushil Kumar, Kumar Raj, Shrivastav Arun, Keshri Amit, Sharma Pankaj
Department of Neurosurgery, SGPGIMS, Lucknow, Uttar Pradesh, India.
J Pediatr Neurosci. 2011 Jan;6(1):78-81. doi: 10.4103/1817-1745.84417.
Esthesioneuroblastoma (ENB) presenting simultaneously with proptosis and bilateral neck metastasis is a very rare presentation. ENB is a rare tumor arising from the olfactory epithelium of the nasal vault which frequently invades the cranial base, cranial vault and orbit. ENB has a bimodal age distribution between 11 and 20 years and between 51 and 60 years. ENB accounts for approximately 1 to 5% of intranasal cancers and no consensus has been reached yet regarding the treatment of this tumor. We are reporting a 17-year-old male patient who presented with right eye proptosis with loss of vision and bilateral neck metastasis. Contrast enhanced computed tomography (CECT) and magnetic resonance imaging (MRI) was done which showed Kadish stage C tumor. Nasal biopsy was done to make the diagnosis and the patient was subjected to radiotherapy. Tumor responded to radiotherapy and both the primary lesion and the cervical lymph node metastasis disappeared. The purpose of this study is to report the rare presentation of proptosis along with bilateral cervical lymph node metastasis in this rare sinonasal tumor.
同时出现眼球突出和双侧颈部转移的嗅神经母细胞瘤（ENB）是一种非常罕见的表现。ENB是一种起源于鼻腔顶部嗅上皮的罕见肿瘤，常侵犯颅底、颅顶和眼眶。ENB的年龄分布呈双峰型，分别在11至20岁以及51至60岁之间。ENB约占鼻内癌的1%至5%，目前对于该肿瘤的治疗尚未达成共识。我们报告一名17岁男性患者，其表现为右眼突出伴视力丧失及双侧颈部转移。进行了增强计算机断层扫描（CECT）和磁共振成像（MRI），显示为卡迪什C期肿瘤。进行了鼻活检以明确诊断，患者接受了放疗。肿瘤对放疗有反应，原发灶和颈部淋巴结转移均消失。本研究的目的是报告这种罕见的鼻窦肿瘤同时出现眼球突出和双侧颈部淋巴结转移的罕见表现。
