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Esthesioneuroblastoma

Esthesioneuroblastoma

Esthesioneuroblastoma (ENB), also called olfactory neuroblastoma, is a rare cancer that occurs in the upper part of the nasal cavity. The disease is believed to arise from the cribriform region of the olfactory nerves that carry the sensation of smell from the nose to the brain.

ENB can develop in all age groups. It accounts for 3-5% of all nasal neoplasms. Although it mainly affects Caucasians, but has been reported in all races. The tumour occurs most commonly in teenagers and in the sixth decade of life.

Most patients with ENB present with symptoms of nasal obstruction causing inability to breath through the involved nostril, nose bleed or both. Rarely, may metastasize to other areas of the body.

Further growth of the tumour can be directed laterally within the orbit resulting in symptoms such as proptosis, extraocular movement paralysis, and blindness or superiorly toward the base of the skull producing intracranial symptoms and complications.


Diagnosis :
Early referral for an intranasal biopsy is key to early diagnosis. A patient with a unilateral nasal obstruction and/or a recurrent epistaxis lasting longer than 1-2 months should be suspected and should undergo a thorough nasal evaluation.

Coronal thin-cut computed tomography (CT) scan is usually the initial radiological study of choice. CT images are essential for correct staging of ENB and should be evaluated carefully for erosion of the cribriform plate, and bone of the skull base.

Magnetic resonance imaging (MRI) is often necessary to better delineate sinonasal, intraorbital or intracranial extension.


Treatment :
The role of an accurate histopathological diagnosis before initiating treatment for ENB is of critical importance. Treatment depends on the tumour stage; the classic treatment strategies for ENB are surgery or radiotherapy or a combination of both. More recently, chemotherapy has been introduced in the therapeutic armamentarium. The optimal treatment sequence varies in each individual case.

Traditionally combined cranio-facial resection is required especially with larger advanced tumours. In this procedure, a skull base surgeon operates through the skull via a craniotomy, while a head and neck surgeon makes the transfacial approach via an incision along the side of the nose (lateral rhinotomy).

Endoscopic endonasal approach has obviated the need for large disfiguring transcranial and transfacial approaches without sacrificing outcomes and with reduced complications.

Olfactory-neuroblastoma Olfactory-neuroblastoma

Olfactory neuroblastoma - Preoperative MRI scan

Olfactory neuroblastoma - Preoperative MRI scan

 

Olfactory-neuroblastoma Olfactory-neuroblastoma

Olfactory neuroblastoma - Postoperative CT scan

Olfactory neuroblastoma - Postoperative CT scan

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Dr.Rayappa Dr. C. Rayappa
Senior Consultant,
Dept. of ENT - Head and Neck Surgery and Skullbase Surgery
Apollo Speciality Hospital, 320, Mount Road, Chennai-600 035, India
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