How Common Is Olfactory Neuroblastoma Or Is It A Rare Disease?

Olfactory neuroblastoma is a very rare cancer that develops in the upper part of the nasal cavity. It is thought to be caused by the nerve tissue associated with the sense of smell. Olfactory neuroblastoma usually grows slowly, but in some cases may develop rapidly and aggressively. Faster growing tumors can spread widely.(1)

How Common Is Olfactory Neuroblastoma Or Is It A Rare Disease?

Olfactory neuroblastoma is a rare malignant tumor of the nasal cavity and paranasal sinuses. It accounts for approximately 6% of nasal and paranasal sinus cancer cases and 0.3% of malignant tumors of the upper digestive tract.(2) To diagnose olfactory neuroblastoma, your doctor will:

  • Ask about any symptoms you have
  • Have a physical examination
  • Require imaging tests (CT and MRI scan) to confirm the presence of the tumor

The diagnostic search for olfactory neuroblastoma is often carried out by an ENT specialist, neurologist, neurosurgeon, and ophthalmologist or in combination. A tumor can be suspected by rhinoscopy. The thick mucus that interferes with the examination is sucked out of the nasal cavity. Rhinoscopy shows a dramatic shift in the nasal septum and the presence of tumor mass, with a reddish-bluish color and a bumpy surface that often bleeds during the inspection. Rhinoscopy makes it possible to distinguish the tumor from other foreign bodies, nasal polyps, and adenoids.

Olfactory neuroblastoma can be detected by x-ray of the paranasal sinuses. Other informative methods include CT of the sinus and MRI of the brain, comprehensive information about the size of the tumor and the limits of its spread can be obtained.

In 70-50% of cases, the tumor cells are detected by cytological analysis of the nasal discharge. However, their absence in a smear does not rule out a tumor. Complete diagnosis of this disease is only possible according to the results of histological examination of tumor tissue and biopsy during rhinoscopy.

With the help of pharynx CT, a search is carried out for regional and distant metastases of olfactory neuroblastoma, neck tissue ultrasound, skeletal scintigraphy, CT of the breast, MRI of the liver and abdomen MSCT.(3)

Symptoms Of Olfactory Neuroblastoma

The most common symptom of olfactory neuroblastoma is chronic congestion on the side of the nose. This can last months or even years before a diagnosis is made.

Other symptoms may include:

Depending on the size and spread of the tumor, you may also encounter:

  • Facial swelling
  • Neurological symptoms
  • Oral and vision problems

Your doctor may refer you to a neurologist or ophthalmologist to check these issues.(4)

Olfactory Neuroblastoma Treatment

Surgery, radiation therapy, or a combination of the two is often used to treat these tumors. Capillary neuroblastoma usually recurs locally and may spread to other parts of the body. The early and aggressive treatment produces the best prognosis.

Minimally Invasive Surgery: Olfactory neuroblastoma can be treated directly using endoscopic nasal endoscopy (EEA). This state-of-the-art minimally invasive method allows surgeons to access the tumor through the natural passage of the nose without the need for an open incision. The surgeon then removes the tumor through the nose and nasal cavity.

The benefits of EEA surgery are healing without incisions, no disfigurement and shortened recovery time. EEA is a suitable treatment option for most olfactory neuroblastomas and has advantages because it produces fewer complications than external methods. If you need complementary therapies, such as radiation or chemotherapy, these can be started immediately after the EEA procedure.(5)

Radiation Therapy And Chemotherapy: Postoperative radiotherapy has been shown to reduce relapse rates.

Chemotherapy can be used for granular blastomas involving relapsed, unresectable or metastatic cases.

Radiation therapy is effective in olfactory neuroblastomas and its regional metastases. It can be administered as proton therapy, intensity-modulated radiation therapy or brachytherapy. Radiosurgery is a special type of radiation therapy. The latter is suitable for the treatment of aesthetics in hard-to-reach places, if, due to the severe somatic condition of the patient, no open surgery can be performed.

Chemotherapy is required if there is a significant spread of cancer and the presence of metastases. It is usually carried out in the preoperative stage of treatment and combined with radiation.(6)

References:

  1. 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.
  2. 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.
  3. Clotman K, Dirinck E, Van Den BJ, et al. A case of severe ectopic adrenocorticotropic hormone syndrome with olfactory neuroblastoma. Endocrine looks can be deceiving. Acta Clinica Belgica. 2016;71(S2):2.
  4. Appukutty S, Di Palma S, Whitaker S, Wood K. Olfactory Neuroblastoma Presenting as a Submandibular Mass. American Journal of Laboratory Medicine. 2019;4(2):35-39.
  5. 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.
  6. Wu J-Y, Chiou J-F, Ting L-L. Chemo-radiotherapy for olfactory neuroblastoma: cases report and literature review. 2019.

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