Olfactory Neuroblastoma (Esthesioneuroblastoma)

What is Olfactory Neuroblastoma?

Overview

Olfactory neuroblastoma, also known as esthesioneuroblastoma, is a rare type of malignant tumor that originates from the specialized nerve cells located in the upper part of the nasal cavity.

These cells are responsible for the sense of smell, hence the name “olfactory” neuroblastoma. Olfactory neuroblastomas (ONBs) are locally invasive tumors that grow into the ethmoid and other air sinuses and can extend onto the orbits as well as the brain.

In cases with intracranial (brain) extension, the tumor typically grows through the anterior skull base dura in the area of the cribiform plate and into the brain cavity elevating or infiltrating the frontal lobes. Spread of disease to local lymph nodes and distant metastases occur in some patients.

The most commonly used grading system for esthesioneuroblastoma is the Kadish system which includes grades A, B and C:

• A. Nasal cavity only
• B. Nasal and paranasal sinus involvement
• C. Extension beyond nasal and paranasal sinus with intracranial, orbital, lymph node or distant metastases

The most common presenting symptoms include nasal congestion, epistaxis (nosebleeds), nasal obstruction, and a decreased sense of smell. Other possible symptoms may include facial pain, headache, vision changes, and swelling around the eyes.

Who is Affected?

Olfactory neuroblastoma primarily affects adults, with a peak incidence in the fifth and sixth decades of life. They occur equally in adult men and women and are uncommon in adolescents.

Olfactory Neuroblastoma Symptomsrn& Causes

Symptoms

Olfactory neuroblastomas typically cause loss of sense of smell (anosmia), epistaxis (nasal bleeding), nasal congestion/obstruction, vision loss and headache. If the tumor invades the dura and intracranial space, seizures and/or cognitive changes may occur.

Causes

The exact cause of olfactory neuroblastoma is not well understood. However, genetic mutations and chromosomal abnormalities are believed to play a role in its development. Exposure to certain environmental factors or occupational hazards may also be associated with an increased risk, although this relationship is not fully established.

When to See a Doctor

Early evaluation and diagnosis can significantly improve treatment options and outcomes. Seek immediate medical attention if you experience:

• Persistent or unexplained nasal bleeding
• Worsening nasal congestion or blockage unresponsive to typical treatment
• Changes in sense of smell or vision
• Persistent headaches or facial pain/swelling
• Neurological symptoms such as seizures or cognitive changes

Olfactory Neuroblastoma Diagnosis

Diagnosing olfactory neuroblastoma requires a combination of imaging studies and histological examination.

Imaging

Imaging techniques such as magnetic resonance imaging (MRI) and computed tomography (CT) scans are used to visualize the tumor, assess its size, extent, and involvement of adjacent structures.

Biopsy

A biopsy is necessary to obtain a tissue sample for microscopic analysis to confirm the diagnosis and determine the tumor grade.

Pre-Operative Angiography and Embolization

For large, highly vascular tumors, pre-operative angiography and tumor embolization is often indicated.

Olfactory Neuroblastoma Treatmentrn& Outcomes

Treatment Options

The aggressive nature of ONB typically warrants multimodality treatment.

Patient Outcomes

With prompt diagnosis and comprehensive treatment, many patients achieve good long-term control of their disease. Prognosis depends on tumor stage at diagnosis, completeness of surgical removal, and response to adjuvant therapy. Long-term follow-up is critical, as these tumors can recur years after treatment.

• Early-stage tumors (Kadish A or B) often have excellent outcomes with combined treatment.
• Advanced-stage tumors (Kadish C) may require more intensive therapy but can still achieve durable control with a multidisciplinary approach.

Managing Olfactory Neuroblastoma

Our team provides personalized care plans, ensuring patients receive comprehensive support throughout their treatment and recovery journey. Living with olfactory neuroblastoma often requires:

Meet our Expert Specialists & Surgeons

Garni Barkhoudarian, MD

Neurosurgery

Jose Carrillo, MD

Neuro-Oncology

Adi Iyer, MD, MS

Neurosurgery, Neuro-interventional Surgery

Daniel F. Kelly, MD

Neurosurgery

Nouzhan Sehati, MD

Neurosurgery

Naveed Wagle, MD

Neuro-Oncology

Walavan Sivakumar, MD

Neurosurgery

Experience Compassionate, Expert Care

By using comprehensive tumor histological subtyping and genomic sequencing, we are able to provide a personalized therapeutic approach for each patient with ONB. We incorporate leading-edge technology and instrumentation with proven surgical experience, making surgery for ONBs and related skull base tumors safer, less invasive and more effective.

Our neurosurgeons work closely with our radiation oncologists, neuro-oncologists and neuropathologists to design the optimal treatment plan that incorporates the latest in radiotherapy techniques. Given advances in personalized and targeted genomic-based therapies, we routinely bank tissue for genetic analysis and detailed tumor biomarker studies. Having such critical biomarker data opens the door for novel therapies and ongoing or future clinical trials.

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