Olfactory neuroblastoma is a malignant tumor that develops from cells of olfactory neuroepithelium; localized in the walls of the nasal cavity, nose of the pharynx and in the ethmoid labyrinth.
It is a rare tumor that makes up 3% of all malignant tumors of the nasal cavity. Only about 1000 cases of the disease have been reported in the world since 1924 when it was first diagnosed. Among malignant neoplasms of the upper respiratory tract, it is of great interest not only because of its relative rarity, but also the features of the morphological structure and clinical course.
According to the morphological structure, these tumors can be distinguished mainly in three types: esthesioneuroblastoma, estesioneurocytoma, and estesioneuroepithelium. Under microscopy, some researchers note typical signs of a neurogenic tumor, such as the formation of rosettes and the presence of membranes with granules similar to tumors of the APUD system.(1)
Can You Die From Olfactory Neuroblastoma?
Predictively, olfactory neuroblastoma prognosis is not very optimistic. Aggressive tumor growth, its germination in the skull and eye sockets, metastasis and recurrence lead to the rapid progression of cancer and death of the patient. Previously, the 5-year survival rate did not exceed 25%.
Lately, thanks to the use of new methods of combined chemoradiation, it has risen to 50-60%. Unfavorable prognostic factors are: over 50 and under 20 years of age, intracranial germination of the neuroblastoma cells, distant metastasis, a high degree of anaplasia of tumor cells.(3)
How Do You Stop Olfactory Neuroblastoma From Spreading?
The treatment method was determined by the size of the tumor focus, as well as sensitivity to chemotherapy. Today, there is no consensus on the methods of treating patients with olfactory neuroblastoma. Some physicians consider it appropriate to perform only surgical interventions, especially in the early stages; others prefer combination therapy with pre- or postoperative radiation therapy, while others consider it sufficient to conduct radiation therapy in an independent version.(5)
Olfactory Neuroblastoma Symptoms
As with other morphological forms of malignant neoplasms of this localization, a general tumor symptom complex very rarely develops with olfactory neuroblastoma. Features of the anatomical and topographic structure of the nasal cavity in children – the narrowness of the nasal passages, small airspace – are usually manifested by a progressive disorder of nasal breathing, up to anosmia.
In this case, there may be mucous and then mucopurulent discharge from the nasal cavity. Further clinical manifestations depend on the direction of tumor growth, its spread to surrounding tissues, and metastasis features. Tumor growth through the cells of the ethmoidal labyrinth towards the orbit causes a restriction of the movements of the eyeball.
The growth of the neoplasm in the posterior parts of the nasal cavity and its spread into the orbit causes exophthalmos, diplopia and other pathological ophthalmological symptoms.(4)
Olfactory Neuroblastoma Diagnosis
Diagnosis of olfactory neuroblastoma is difficult, especially in the early stages. Initially, the neoplasm is taken as an inflammatory process, adenoids, a foreign body and polyps of the nasal cavity.
Therefore, children undergo prolonged inadequate therapy, usually including physiotherapy. As a rule, the development of a tumor in the nasal cavity is accompanied by hypersecretion of viscous mucus, which complicates a detailed examination. Thus, it is necessary to sanitize the general nasal passage using an electric aspirator and then proceed with the study.
With anterior rhinoscopy in the upper and rear sections of the nasal cavity, it is possible to detect a tumor mass. With instrumental palpation, a dense-elastic tumor is felt, sometimes bleeding after the manipulation.
An X-ray examination of the sinuses and nasopharynx is often informative enough to establish the fact of the tumor, but additional projections must be used to determine the extent and its spread. The most informative diagnostic methods for tumors of the nasal cavity and paranasal sinuses at this stage are CT and MRI.(5)
Olfactory Neuroblastoma Pathogenesis
Occurring mainly in adults, olfactory neuroblastoma is much less studied in childhood. But the tumor develops equally often in children of both sexes, mainly after the age of 10 years. At the initial stages, it is usually localized in the upper parts of the nasal cavity.
Possessing aggressive locally-destructive growth, the tumor spreads and fills the entire half of the nasal cavity, grows into the paranasal sinuses, base and cranial cavity. It is capable of extensive regional and distant metastasis.
In childhood, metastases to regional lymph nodes and relatively distant metastases are relatively less common. The regional lesion area is the pharyngeal, upper deep cervical, parotid and submandibular lymph nodes.(2)
- Abdelmeguid AS, Teeramatwanich W, Roberts D, et al. Long-Term Outcomes of Management of Olfactory Neuroblastoma: MD Anderson Experience. Journal of Neurological Surgery Part B: Skull Base. 2018;79(S 01):A184.
- 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.
- Song X, Wang J, Wang S, Yan L, Li Y. Prognostic factors and outcomes of multimodality treatment in olfactory neuroblastoma. Oral Oncology. 2020;103:104618.
- 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.
- 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.
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- How To Diagnose Olfactory Neuroblastoma & What Is The Best Medicine For It?