What Are The Ways To Prevent Olfactory Neuroblastoma & Does It Reoccur?

Neuroblastoma is a type of cancer. Olfactory neuroblastoma is a rare tumor that originates in the olfactory nerve in the nasal cavity. Diagnosis is difficult, and there are many cases where the diagnosis varies from hospital to hospital 1.(1)

This malignant tumor particularly develops at the level of neuroblasts, which are immature nerve cells of the sympathetic nervous system. The latter constitutes one of the three pillars of the autonomic nervous system which directs the involuntary functions such as respiration and digestion.

What Are The Ways To Prevent Olfactory Neuroblastoma?

Although no cancer prevention method has been proven to prevent it from developing, the best prevention method to prevent olfactory neuroblastoma is to stop smoking. Annual visits to ENT specialties are also the best way to diagnose early or prevent the development of olfactory neuroblastoma.(3)

Onset Rate And Age Of Onset Of Neuroblastoma

Statistically, 1 in 7,000 births (0.007%) of babies is born with this disease. About 90% of affected children are under 5 years of age, of which about 50% are under 2 years of age. 0-year-olds have the highest incidence by age, followed by 3-year-olds. Each year, about 200 children are diagnosed with neuroblastoma.

Neuroblastoma can develop in different regions of the body. However, this type of cancer most often appears in the abdomen at the level of the adrenal glands (located above the kidneys), as well as along the spine. More rarely, it can occur in the neck, chest or pelvis (small pelvis).

While developing, neuroblastoma can cause metastases. These are secondary cancers: the cells of the primary tumor escape and will colonize other tissues and organs.

Most olfactory neuroblastomas begin in the nasal cavity and ethmoid sinus. The disease can occur in a wide range of ages, from young to elderly, but its peak is relatively high in people in their 20s and 60s. There is no major difference in gender frequency.(2)

Does Olfactory Neuroblastoma Reoccur?

Surgery is usually used to manage tumors apart from radiation therapy or a combination of both. The disease often recurs locally and may also spread to other parts of the body. Early and quick interventions result in better prognosis.(5)

Symptoms

Olfactory neuroblastoma is initially asymptomatic, but the main symptom is congestion in the nose.

However, patients with nasal congestion alone are less likely to trigger a visit to the hospital, and a few months after the manifestation of the nasal congestion, when a patient is first consulted after feeling uneasy about frequent and difficult-to-stop epistaxis, the patient usually visit a doctor.

Causes Of Neuroblastoma

It is thought that the cause of neuroblastoma is the cells that differentiate into nerve cells (neuroblasts) becoming malignant and developing into solid tumors with time.

Normally, neuroblasts stop growing around three months after birth. But some abnormality causes tumors to grow without stopping. However, the cause of this phenomenon has not been identified to date. The probability of inheritance of this disease is low.

How To Diagnose?

The diagnosis of neuroblastoma consists of defining the type of neuroblastoma and in evaluating its evolution. This diagnosis is made by several exams including:

The use of medical imaging techniques such as an ultrasound, a CT scan or even MRI

A tumor biopsy (analysis of the tumor tissue)

Beyond these examinations, the diagnosis will also seek to identify the presence of metastases, the secondary cancers that can develop from the original tumor. It is considered that 50% to 60% of patients have metastases. For this, a bone marrow assessment is performed to assess the rate of metabolites of urinary catecholamines. This measurement makes it possible to confirm the presence of metastases.(4)

Treatment

Standard treatment for olfactory neuroblastoma is complete surgical resection and postoperative radiation therapy. Chemotherapy is the mainstay of treatment for unresectable cases and distant metastases. There are no established treatments at this time, but studies have shown that some patients have benefited from cisplatin-based chemotherapy (such as cisplatin plus etoposide or cisplatin plus irinotecan) that is similar to lung cancer chemotherapy.

However, no treatment has been established due to the very small number of patients. Depending on the facility, stereotactic radiotherapy with a higher dose of radiation at one time, new radiotherapy such as particle beam irradiation, and multidisciplinary treatment using different chemotherapy is performed.(5)

References:

  1. Wu J-Y, Chiou J-F, Ting L-L. Chemo-radiotherapy for olfactory neuroblastoma: cases report and literature review. 2019.
  2. Harvey RJ, Nalavenkata S, Sacks R, et al. Survival outcomes for stage‐matched endoscopic and open resection of olfactory neuroblastoma. Head & neck. 2017;39(12):2425-2432.
  3. Peckham M, Wiggins R, Orlandi R, Anzai Y, Finke W, Harnsberger H. Intranasal esthesioneuroblastoma: CT patterns aid in preventing routine nasal polypectomy. American Journal of Neuroradiology. 2018;39(2):344-349.
  4. Kaufmann MR, Camilon PR, Janz TA, Levi JR. Factors Associated With the Improved Survival of Head and Neck Neuroblastomas Compared to Other Body Sites. Annals of Otology, Rhinology & Laryngology. 2019;128(3):241-248.
  5. Yin Z, Wang Y, Wu Y, et al. Age distribution and age-related outcomes of olfactory neuroblastoma: a population-based analysis. Cancer management and research. 2018;10:1359.

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