Abstract
Background: Olfactory neuroblastoma also known as Esthesioneuroblastoma (ENB) is a tumor arising from the basal layer of olfactory epithelium in the superior recess of the nasal cavity in the region of cribriform plate. Incidence peaks once in 11 - 20 years of age and again in 50 - 60 years of age. It is equally found in men and women. Aim: The main aim of this case report is to characterize the clinical features of ENB showing nasopharyngeal involvement and its importance in the differential diagnosis of sinonasal neuroendocrine malignancies. Case presentation: Two cases are reported. The first case was a 21-year-old male with symptoms of nasal obstruction, recurrent epistaxis, nasal discharge, headache and paresthesia over the face with an evolution of 1 year with no previous history of trauma. Diagnostic nasal endoscopy revealed reddish gray mass filling bilateral nasal cavities. Posterior rhinoscopy revealed mass coming through choanae and filling the nasopharynx. Endoscopic resection of tumor was done and postoperatively all the symptoms of the patient resolved. The second case was a 62-year-old male with complaints of nasal obstruction, nasal discharge, recurrent epistaxis, anosmia and difficulty in swallowing. On computed tomography, anteriorly, the mass was extending up to external nares and posteriorly up to the nasopharynx, superiorly up to nasal roof and inferiorly up to hard palate. No obvious erosion of floor of anterior cranial fossa was seen. No intracranial extension was seen. Surgery was advised but patient refused to undergo any treatment. Conclusion: This study highlights the characteristics and clinical features of ENB showing nasopharyngeal involvement and their importance in the differential diagnosis of sinonasal neuroendocrine malignancies.
Highlights
Olfactory neuroblastoma is an uncommon tumor of neuroectodermal origin, arising from basal cells of the olfactory neuroepithelium [1] [2]
Olfactory neuroblastoma represents less than 5 percent of all sinonasal malignancies
Esthesioneuroblastoma originates from olfactory epithelium in the upper nasal cavity in the region of the cribriform plate
Summary
Olfactory neuroblastoma is an uncommon tumor of neuroectodermal origin, arising from basal cells of the olfactory neuroepithelium [1] [2]. Olfactory neuroblastoma represents less than 5 percent of all sinonasal malignancies. It is histologically similar to adrenal or sympathetic ganglionic neuroblastomas and retinoblastomas. The incidence of this tumor has a bimodal distribution with peaks at 20 and 50 years of age [3]. The non-specific symptoms of nasal obstruction, recurrent epistaxis, hyposmia, headache and the special anatomical location of the tumor often lead to a diagnosis of benign paranasal disease delaying the correct diagnosis [4]. It tends to spread submucosally in all directions to involve the paranasal sinuses, nasal cavity, oral cavity, the orbits, and the brain [5] [6]. Neuro endocrine tumor is capable of causing paraneoplastic syndromes by secreting peptides
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