What is Esthesioneuroblastoma (Olfactory Neuroblastoma)?
Esthesioneuroblastoma, also known as olfactory neuroblastoma, is a rare type of cancer that starts in the sinuses, specifically from the cells responsible for our sense of smell. It usually occurs in the upper part of the nasal cavity. This cancer was first described in 1924 by Berger and Luc, and since then, over a thousand cases have been reported globally. Esthesioneuroblastoma is known for growing and spreading aggressively. It can spread through both the bloodstream and the lymph system, which is part of the body’s immune system.
What Causes Esthesioneuroblastoma (Olfactory Neuroblastoma)?
The precise cause of a condition known as esthesioneuroblastoma isn’t fully understood yet. Similarly, the factors that might make a person more likely to get this condition are also unknown.
Risk Factors and Frequency for Esthesioneuroblastoma (Olfactory Neuroblastoma)
Esthesioneuroblastoma is a rare type of cancer that affects the nasal cavity and paranasal sinuses. It accounts for only a small percentage of all upper aerodigestive tract malignancies, which are cancers that occur in the upper part of the digestive tract and airway. However, the number of new cases has been increasing over the past decade. This disease can develop in people of any age, but it’s most commonly seen in young adults. There’s also a second peak occurrence in later adulthood. Interestingly, it affects both men and women evenly.
- Esthesioneuroblastoma is a rare form of cancer that takes place in the nasal cavity and paranasal sinuses.
- It makes up 2% to 6% of all cases of nasal and paranasal sinus cancers, and 0.3% of all upper aerodigestive tract cancers.
- The incidence of this cancer has been rising in the past decade.
- Esthesioneuroblastoma can occur at any age, but it’s commonly seen in young adults.
- There’s a second increase in incidence in later adulthood.
- It affects both men and women equally.
Signs and Symptoms of Esthesioneuroblastoma (Olfactory Neuroblastoma)
Esthesioneuroblastoma, a type of nasal tumor, often has symptoms that can be easily mistaken for a simple infection or inflammation. This often results in delayed diagnosis. People suffering from this condition usually experience nasal blockage and nosebleeds early on. However, depending on where the tumor is and how much it has spread, other symptoms can also develop. For instance, some individuals might lose their sense of smell years before the tumor is detected.
If the tumor extends into the surrounding areas, it can cause various other symptoms such as:
- Facial swelling or a mass near the inner corner of the eye from spreading to the paranasal sinuses
- Eye-related issues like pain, bulging eyeballs, excessive tearing, or vision problems if it spreads to the eye socket
- Headaches if it reaches the front part of the skull’s interior
- Problems linked to the overproduction of antidiuretic hormone
Testing for Esthesioneuroblastoma (Olfactory Neuroblastoma)
If a doctor suspects that you might have a tumor in your sinus, a series of tests may be carried out to help confirm the diagnosis.
The first step is usually a complete physical examination that includes an extensive check of your neurological, eye, and head and neck health. They may also use a flexible fiber-optic endoscope, which is a device that allows the doctor to view the inside of your sinus.
A computed tomography (CT) scan may be ordered as part of the diagnostic process. This is a type of X-ray that produces detailed images of the body. In this case, a contrast agent would be used, which helps highlight areas in your sinus. This type of test is particularly useful to look for sinus disease and areas where the bone may be damaged.
They may also recommend a magnetic resonance imaging (MRI) scan. The MRI uses magnetic fields and radio waves to produce detailed images of the body. This particular test is useful in differentiating between a tumor and other secretions, and it helps doctors to examine in detail the area around the eyes and brain.
There’s also a specific nuclear medicine scan that could be used. This type of scan uses a small amount of radioactive material that would make the tumor glow during the scan, helping doctors identify it and differentiate it from other types of tumors.
Finally, doctors might recommend a biopsy of the tumor. This involves taking a small sample of the tumor under general anesthesia for further examination. The biopsy is typically done after the imaging studies are conducted to learn more about the tumor, such as its blood supply.
Treatment Options for Esthesioneuroblastoma (Olfactory Neuroblastoma)
There are three main treatment options for a type of cancer called esthesioneuroblastoma. These include surgery, radiation therapy (using high-energy beams to destroy cancer cells), and chemotherapy (using drugs to kill cancer cells). Many times, a combination of these treatments is used.
For small tumors that have not spread to other parts of the body, surgical removal is the preferred way to go. But as the disease gets more advanced, doctors usually suggest more extensive when the cancer has spread further. This could include procedures like an open or endoscopic-assisted craniofacial resection (a surgery that involves removing parts of the face and skull) along with radiation therapy after the surgery.
It’s also possible to do radiation before the surgery, which can yield similar effects. Chemotherapy can enhance the effects of post-surgery radiation in controlling the spread of the disease.
For patients where the disease has spread to distant parts of the body, relieving symptoms and improving quality of life, or ‘palliative therapy’, might be the best option. If the cancer can’t be removed surgically because it’s spread too much, chemoradiation (a mix of chemotherapy and radiation treatment) can sometimes be used to make surgery possible in previously inoperable cases.
What else can Esthesioneuroblastoma (Olfactory Neuroblastoma) be?
The types of malignant tumors (cancer) include:
- Extramedullary plasmacytoma
- Extraosseous Ewing sarcoma/Primitive neuroectodermal tumor (PNET)
- Mesenchymal chondrosarcoma
- Natural killer/T-cell lymphoma
- Rhabdomyosarcoma
- Sinonasal malignant melanoma
- Sinonasal neuroendocrine carcinoma
- Sinonasal undifferentiated carcinoma
- Small cell osteosarcoma
- Small cell undifferentiated (neuroendocrine) carcinoma
- Synovial sarcoma
- Undifferentiated (lymphoepithelioma like) carcinoma
What to expect with Esthesioneuroblastoma (Olfactory Neuroblastoma)
The outlook for people with a type of cancer called esthesioneuroblastoma depends on both the stage and grade of the cancer. In simpler terms, this means how advanced the cancer is and how aggressively it’s behaving.
For example, if the tumor is at stage A (a very early stage of cancer), there’s a 75% to 90% chance of living for at least five years after diagnosis. In comparison, for stage C tumors (a more advanced stage), the 5-year survival rate drops to 45%. The grade of the tumor also matters – high-grade tumors, which are more aggressive, have a 25% 5-year survival rate, while low-grade tumors, which are less aggressive, have an 80% survival rate.
Factors that could lead to a worse outcome for a person diagnosed with esthesioneuroblastoma include: having cancer that has spread to other parts of the body (metastases), being in a high stage or having a high-grade tumor, being above 50 or below 20 at the time of diagnosis, the cancer spreading to the brain (intracranial spread), the cancer cells growing rapidly (high proliferation), being female, or having cells that have an abnormal number of chromosomes (called polyploidy or aneuploidy).
Possible Complications When Diagnosed with Esthesioneuroblastoma (Olfactory Neuroblastoma)
Esthesioneuroblastoma, a type of cancer, commonly returns within two years of first appearing, affecting about 30% of patients. Additionally, the disease spreads in 35% of cases, frequently targeting the cervical lymph nodes. About 10% of patients experience distant metastasis, in which the cancer travels to distant parts of the body such as the lungs, bone, liver, or skin.
Key Facts:
- Esthesioneuroblastoma often returns within the first 2 years in about 30% of patients
- The disease spreads in roughly 35% of cases
- The cervical lymph nodes are a usual site of spread
- Distant metastasis, or spread of the cancer to far parts of the body, occurs in around 10% of patients
- Metastasis commonly targets the lungs, bone, liver, and skin
Preventing Esthesioneuroblastoma (Olfactory Neuroblastoma)
It’s important for patients and their families to learn how to identify the early signs and symptoms of tumors in the nose and sinus area, also known as sinonasal tumors. If a patient experiences symptoms like blocked nose, nosebleeds, or loss of smell, they should seek medical attention quickly. This early detection is key in the treatment of a type of cancer known as esthesioneuroblastoma. By responding to these signs early and getting to a hospital promptly, there’s a higher chance of successful treatment and recovery.
