Abstract
Objectives:To present the clinicopathological experience of Olfactory Neuroblastoma (ONB) with emphasis on histopathological and immunohistochemical features.Methods:A descriptive cross-sectional study was done on 36 cases of ONB, selected by non-probability purposive sampling. Theses cases of ONB were retrieved and reviewed from surgical pathology database of Aga Khan University Hospital reported between January 1993 and March 2015.Results:Tumor size and age of presentation was wide in range without any distinct bimodal distribution. Nasal cavity was most common site along with involvement of paranasal sinuses. More than 50% cases had Kadish stage A. Microscopically, most cases were Grade-1 and majority showed partial or complete lobular architecture. Neurofibrillary matrix was observed in 2/3rd of cases. Among immunohistochemical markers, Neuron Specific Enolase was most frequently expressed. Unusual positive expression of Cytokeratin AE1/AE3 and Cytokeratin CAM5.2 was also seen focally in few cases.Conclusion:The ONB has great variability of histological and clinical presentation, and immunohistochemical markers are useful to differentiate from more common small round blue cell tumours of nasal cavity.
Highlights
Olfactory neuroblastoma (ONB), known as Esthesioneuroblastoma, is a rare but distinct malignant neuroectodermal tumor arising from the olfactory epithelium of roof of nasal cavity and cribriform plate
But cases primarily arising from other parts of nasal cavity, paranasal sinuses and frontal lobe of brain have been reported.[2]
H&E stained microscopic glass slides were reviewed by two pathologists and were analyzed for various histological features including growth pattern, architecture, neurofibrillary matrix, rosettes, nuclear pleomorphism, nucleoli, mitotic activity, necrosis and calcification
Summary
Olfactory neuroblastoma (ONB), known as Esthesioneuroblastoma, is a rare but distinct malignant neuroectodermal tumor arising from the olfactory epithelium of roof of nasal cavity and cribriform plate. It account for 2 to 3% of intranasal tumors.[1]. But cases primarily arising from other parts of nasal cavity, paranasal sinuses and frontal lobe of brain have been reported.[2] These tumors are associated with a number of symptoms including nasal obstruction, epistaxis, headaches, visual disturbances, proptosis, and anosmia.[3,4]. Typical morphologic features of these tumors have been described but most
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