What is an Olfactory Neuroblastoma aka Esthesioneuroblastoma?

Olfactory neuroblastoma
Olfactory neuroblastoma. Grade 1 olfactory neuroblastoma arranged in lobules with abundant neurofibrillary matrix and a rim of flattened sustentacular cells at the periphery of lobules. (H&E stain 200x magnification) Grade 4 olfactory neuroblastoma arranged in pattern-less sheets of markedly pleomorphic tumor cells. (H&E stain 200x magnification) Tumor cells invading into the brain tissue. (H&E stain 200x magnification). Olfactory neuroblastoma: A clinicopathological experience of a rare entity from Pakistan: Haroon S, Tariq MU, Memon A, Fatima S, Hasan SH - Pakistan journal of medical sciences (2016 May-Jun). Not Altered. CC.

Olfactory Neuroblastoma aka Esthesioneuroblastoma is rare, highly malignant tumor composing small round cells similar to neuroblasts thriving into lobular nests which are surrounded by vascularized connective tissue.

What is the Pathology of Olfactory Neuroblastoma aka Esthesioneuroblastoma?

The pathology of olfactory neuroblastoma aka esthesioneuroblastoma is:

-Etiology: The cause of olfactory neuroblastoma aka esthesioneuroblastoma is, has no clear exposure or etiologic agent  

-Genes involved: Drosophila achaete-scute gene (hASH1).

-Pathogenesis: The sequence of events that lead to olfactory neuroblastoma aka esthesioneuroblastoma. Tumor cells are mitotically dynamic and they are forerunner cells that progress into neuronal and sustentacular cells.

-Histology: The histology associated with olfactory neuroblastoma aka esthesioneuroblastoma shows neoplastic cells displaying more diffuse growth patterns, cytological atypia of the neoplastic cells. The neuroblasts that rise mostly superiorly, laterally of the nose from the neuroendocrine cells disseminated at the olfactory mucosa.

How does Olfactory Neuroblastoma aka Esthesioneuroblastoma Present? 

Patients with olfactory neuroblastoma aka esthesioneuroblastoma typically mark males and females similarly present at age range of 3-90years. The symptoms, features, and clinical findings associated with olfactory neuroblastoma aka esthesioneuroblastoma include Nasal discharge, obstruction, unilateral polyp, epistaxis, anosmia, paralysis of extraocular movement, and blindness headache, cervical – mass, nausea.

How is Olfactory Neuroblastoma aka Esthesioneuroblastoma Diagnosed? 

Olfactory neuroblastoma aka esthesioneuroblastoma is diagnosed by biopsy. 

 How is Olfactory Neuroblastoma aka Esthesioneuroblastoma Treated? 

Olfactory neuroblastoma aka esthesioneuroblastoma is treated through chemotherapy, radiation. Surgical, endoscopic craniofacial resection.

What is the Prognosis of Olfactory Neuroblastoma aka Esthesioneuroblastoma?

The prognosis of olfactory neuroblastoma aka esthesioneuroblastoma good though a subset displays recurrence, advanced and/or metastatic ailment, requiring additional management.