Esthesioneuroblastoma presenting with proptosis and bilateral neck metastasis: An unusual presentation

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.