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Olfactory Neuroblastoma - Causes, Symptoms, Grades, and Management

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Olfactory neuroblastoma is an uncommon cancerous growth that involves the nasal epithelial tissue and nerves. It is also known as “Esthesioneuroblastoma.”

Written by

Dr. Ruchika Raj

Medically reviewed by

Dr. Akshay. B. K.

Published At October 14, 2022
Reviewed AtMarch 17, 2023

Introduction:

Olfactory neuroblastoma is a very rare tumor that involves the nasal tissues, olfactory nerves (nerve supplying to nose), and cribriform plate and can also spread to other body parts like lungs, skull bones, brain, and neck. It is also called “olfactory carcinoma” as the cancer is derived from neuroectodermal olfactory cells that are present in the upper part of the nasal cavity, cribriform plate of the ethmoid, nasal septum, and roof of the nose. Olfactory nerves damaged by the tumor cause loss of smell sensation in people. Olfactory carcinoma can occur at any age but are more common in adults.

What Is the Prevalence of Olfactory Neuroblastoma?

It is a rare type of cancer that accounts for three to four percent of total nasal tumors.

What Is the Incidence of Olfactory Neuroblastoma?

It affects one in 2.5 million people every year and is more common in people in the age group of 50 to 70 years old.

What Are the Causes of Olfactory Neuroblastoma?

The exact cause of olfactory neuroblastoma is still unknown. But it is found by research that it is caused by abnormal alteration of parts of DNA (deoxyribonucleic acid), mostly involving chromosome 2, chromosome 5, chromosome 6, chromosome 7, and chromosome 20.

What Are the Signs and Symptoms of Olfactory Neuroblastoma?

Various signs and symptoms related to olfactory carcinoma are:

  • Loss of smell sensation.

  • Pain around the eyes.

  • Epistaxis (bleeding from the nose).

  • Pus drainage from the nose.

  • Headaches.

  • Blurred vision.

  • Difficulty in breathing.

  • Nose blockade.

  • Postnasal drip (nasal drainage in the throat)

  • Swelling near eyes, nose, and face.

What Are the Different Grades of Olfactory Neuroblastoma?

Olfactory neuroblastoma is classified into four grades:

  • Grade A: When the tumor involves only the nasal cavity.

  • Grade B: When the tumor involves the nasal cavity, along with all the paranasal sinuses (Sinus or hollow cavity present lateral to the nose).

  • Grade C: When the tumor involves the nasal cavity and paranasal sinus and also spreads to the cribriform plate, intracranial spaces, and skull base.

  • Grade D: Along with areas of Grade C, it also involves lymph nodes of the neck and spreads to other parts of the body like the lungs, liver, and brain.

How Can We Diagnose Olfactory Neuroblastoma?

Diagnostic aids for olfactory neuroblastoma are:

  • Clinical Examination and History: Complete clinical examination of the nasal cavity is done to check for swelling, draining pus from the nose that is collected and sent to the lab for investigations. History of symptoms like loss of smell, watery eyes, and pain/tenderness near the eyes should be noted.

  • Computed Tomography (CT) Scans: These scans are done to check the size, location, and extent of the tumor spread. The presence of a dumbbell shaped tumor across the cribriform plate (part of ethmoid bone that forms the roof of the nasal cavity) is one of the characteristic findings observed in the scan.

The unique dumb-bell shape of the tumor mass is formed due to the presence of the upper mass of the tumor in the intracranial fossa (skull bone cavity) and the lower mass of the tumor in the nasal cavity, and the middle point is formed by the cribriform plate.

  • Magnetic Resonance Imaging (MRI): These scans help to check the spread of cancer in various areas of the skull, eyes, sinus cavities, and brain. A contrast media like gadolinium is used before doing the scan to enhance the visibility of the areas of spread.

  • Biopsy: A sample of small tissue is taken from the tumor and sent to the lab to confirm the presence of olfactory carcinoma.

  • Positron Emission Tomography (PET) Scan: A radioactive material is injected through the vein, and three-dimensional scans are taken. It is used to assess the involvement of the lymph nodes of the neck and the spread of cancer to various areas of the body. It also helps to grade cancer based on the diagnosis.

What Is the Treatment Plan For Olfactory Neuroblastoma?

Various treatment modalities for olfactory carcinoma are:

  • Radiotherapy: Radiation therapy is given to the patient to kill the cancer cells and prevent their recurrence.

  • Surgical Removal of the Tumor: The tumor mass is removed surgically with some amount of healthy tissue margins so that all tumor cells are removed for safety to prevent the recurrence of the tumor. It is done by either nasal endoscopic surgery or open craniotomy resection, depending on the extent of the tumor.

  • Chemotherapy: Chemotherapeutic agents such as Cisplatin and Carboplatin are prescribed to the patient along with radiation therapy, and after the surgery is done in advance if the cancer is spread to other parts of the body.

Can Olfactory Neuroblastoma Recur After Surgery?

Yes, although the surgery completely removes the tumor mass, any cancer cell behind can lead to the recurrence of the tumor. Post-surgical radiotherapy and chemotherapy play a tremendous role in the prevention of the recurrence of the tumor, especially in grade C tumor cases where cancer has involved lymph nodes of the neck and other body parts like skull bones, lungs, and brain.

What Are the Complications After Surgery for Olfactory Neuroblastoma?

Complications that can occur after surgery are:

  • CSF rhinorrhea (cerebrospinal fluid leak from the nasal cavity).

  • Hematoma (collection of blood between the tissue spaces).

  • Loss of smell.

  • Recurrence of the tumor.

  • Change in the facial aesthetics (look of the face changes after removal of the damaged bone).

Conclusion:

Olfactory neuroblastoma is a very rare tumor and can affect anyone during adulthood. Symptoms related to olfactory carcinomas like loss of smell, watery eyes, difficulty in breathing through the nose, and nasal discharge should be noticed and reported to the doctor immediately for diagnosis and management of the tumor in the early stage for a satisfactory outcome of the treatment. The tumor that is not diagnosed early and advances to grade C makes the treatment very complex for both the patients and the doctor, as the outcome of the treatment is not good because of the spread of cancer to different areas of the body.

However, there are various treatment modalities available for an advanced grade of olfactory neuroblastoma, but the risk of recurrence is more in such cases, so post-surgical follow-up with the doctor for radiation therapy and chemotherapy is crucial to prevent the recurrence of the tumor in advanced cases.

Frequently Asked Questions

1.

What Is the Survival Rate of Olfactory Neuroblastoma?

Doctors determine the survival rates of olfactory neuroblastoma by assessing the historical outcomes of groups of individuals diagnosed with this condition. The survival rate is known to fluctuate based on the stage of the disease, with estimates ranging from 50 percent to 90 percent.

2.

Is Olfactory Neuroblastoma Classified as Brain Cancer?

It is true that olfactory neuroblastoma falls under the category of brain cancer. It is an uncommon type of malignant tumor that develops near the brain in the upper region of the nasal cavity. Olfactory neuroblastoma is a form of sinonasal cancer that might be difficult to cure due to its closeness to the brain.

3.

What Are the Stages Involved in Olfactory Neuroblastoma?

The staging system for olfactory neuroblastoma categorizes the disease into three stages:
- Stage A: The tumor is limited to the nasal cavity.
- Stage B: Tumors have extended beyond the nasal cavity and invaded the paranasal sinuses surrounding the nose.
- Stage C: Cancer has progressed further, spreading from the nasal cavity and paranasal sinuses into nearby tissues.

4.

What Are the Characteristic Features of Olfactory Neuroblastoma?

Olfactory neuroblastoma is a rare, slowly progressing malignancy that develops from olfactory nerve cells in the nasal cavity. It can result in eye symptoms like proptosis (bulging eyes)and double vision as well as nasal symptoms like obstruction and epistaxis (nosebleed). Due to the tumor's potential to penetrate adjacent structures and initial asymptomatic character, early diagnosis might be difficult.

5.

Is Olfactory Neuroblastoma Curable?

Esthesioneuroblastoma, another name for olfactory neuroblastoma, may be cured. The size and stage of the tumor at diagnosis are just two of the variables that affect how well a patient responds to treatment. The likelihood of a successful outcome and long-term survival can be increased by early detection and vigorous treatments like radiation and surgery. To check for probable recurrence, regular follow-up care is crucial.

6.

What Are the Treatment Options for Olfactory Neuroblastoma?

Esthesioneuroblastomas or olfactory neuroblastoma are typically treated with surgery, radiation therapy, or a combination of both. However, it is important to note that these tumors often recur in the same location and can potentially spread to other areas of the body. Early and aggressive treatment approaches have been shown to offer the most favorable prognosis in managing this condition.

7.

Is Stage 4 Neuroblastoma Potentially Curable?

Overall, there has been a notable improvement in the chances of cure for our stage 4 neuroblastoma patients over time. The survival probability has reached an encouraging rate of 89.7 percent, which closely aligns with the rates reported in the recent series. When comparing the outcomes of patients from the first two eras, their overall survival (OS) rates were very similar, with 76.9 percent for the first era and 77.2 percent for the second era. This progress demonstrates significant advancements in the management and treatment of stage 4 neuroblastoma, leading to higher chances of survival for affected patients.

8.

What Is the Origin of Olfactory Neuroblastoma?

ONB, also known as olfactory neuroblastoma, is believed to originate from specialized sensory neuroepithelial (neuroectodermal) olfactory cells primarily located in specific regions of the nasal cavity. These regions include the superior nasal concha, the upper part of the septum, the nasal roof, and the cribriform plate of the ethmoid. Early detection and appropriate medical intervention are crucial for the effective management and treatment of this condition.

9.

How Is Neuroblastoma Confirmed as a Diagnosis?

Neuroblastoma commonly metastasizes to the bone marrow, affecting the soft inner parts of specific bones. Elevated levels of catecholamines in blood or urine can serve as indicators, and the presence of cancer cells in a bone marrow sample may be sufficient for diagnosing neuroblastoma, potentially eliminating the need for a biopsy of the primary tumor.

10.

Which Chemotherapy Is Used in the Treatment of Olfactory Neuroblastoma?

Neoadjuvant therapy with a combination of Etoposide, Ifosfamide, and Cisplatin has been explored for the treatment of olfactory neuroblastoma. Additionally, a retrospective analysis of 12 cases has shown that a non platinum-based chemotherapy regimen, combining Irinotecan and Docetaxel, is being investigated for advanced or metastatic olfactory neuroblastoma.

11.

Is Neuroblastoma Curable in General?

Neuroblastoma treatment is based on risk groups. Age, tumor histology, and tumor biology are other factors. Low risk, intermediate risk, and high risk are the three categories of risk. There is a good possibility of curing neuroblastoma that is low-risk or intermediate-risk. Neuroblastoma at high risk may be difficult to treat.

12.

Can Damaged Olfactory Nerves Undergo Healing?

Yes, olfactory nerve injury is capable of partially recovering. The olfactory nerve has some ability to regenerate, enabling it to heal and regain function after harm. However, the degree of recovery may differ based on the magnitude of the injury and the person's general health. The olfactory function may not always completely recover.

13.

How Long Does It Usually Take for Olfactory Nerves to Repair?

The time required to restore the olfactory nerve varies greatly and might range from several weeks to months. While in less severe injuries, obvious improvements may not be seen for several months, in more severe injuries, partial or complete recovery of olfactory function may happen within a few weeks. But occasionally, the olfactory nerves may not completely recover, resulting in a long-lasting or irreversible loss of smell.

14.

Is Neuroblastoma a Painful Condition?

The spread of neuroblastoma to bones is frequent. A child who can speak might express bone pain. The child may walk with a limp or refuse to walk because of the pain. Tumors that spread to the spine's bones might press on the spinal cord, resulting in weakness, numbness, or paralysis in the arms or legs.

15.

Does Neuroblastoma Have a Tendency to Spread Rapidly?

Yes, neuroblastoma has a tendency to spread rapidly. It is known for its aggressive metastatic behavior, often spreading to distant sites such as the bones, bone marrow, lymph nodes, and other organs in a short period of time. Early diagnosis and intervention are essential to improve the prognosis and treatment outcomes for affected children.

16.

How Many Rounds of Chemotherapy Are Typically Administered for Neuroblastoma?

Surgery plays a crucial role in treating children at intermediate risk, but it is seldom sufficient as a standalone treatment. In most cases, children undergo four to eight cycles of chemotherapy, lasting approximately 12 to 24 weeks, either before or after the surgical procedure. This combined approach aims to enhance the effectiveness of the treatment and improve the overall outcomes for the patients.

17.

What Is the Underlying Cause or Origin of Neuroblastoma?

The majority of neuroblastomas are caused by gene alterations in neuroblasts that take place during a child's development, perhaps even before birth. It is unknown what causes these acquired gene alterations. It is possible that they are merely random cellular occurrences that occasionally take place without any external cause.
Dr. Akshay. B. K.
Dr. Akshay. B. K.

Otolaryngology (E.N.T)

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nasal and sinus cancerolfactory neuroblastoma
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