Olfactory neuroblastoma is a rare disease that is said to originate from the olfactory nerve in the nasal cavity. Diagnosis is difficult, and there are many cases where the diagnosis varies from hospital to hospital. Different reasons make it difficult to diagnose olfactory neuroblastoma and its symptoms.(1)
Tumors derived from neuroendocrine cells are collectively called neuroendocrine tumors (NET). It occurs mostly in the digestive organs such as the pancreas and stomach and the lungs, but also the nasal cavity, parathyroid gland, and adrenal glands.(2)
What Happens To Untreated Olfactory Neuroblastoma?
You might be unable to connect the nose with cancer. However, a few individuals may have a form of carcinoma in the nasal cavity known as olfactory neuroblastoma. As with most types of cancer, it can be fatal if left untreated. Moreover, the cancer cells can spread to other parts of the body causing cancer to different organs.
Diagnosis Of Olfactory Neuroblastoma
The tissue is removed from the tumor and pathologically diagnosed. The extent of the disease can be ascertained by imaging tests such as fiberscope and CT as well as MRI. Neuroendocrine tumors of the nasal cavity, including olfactory neuroblastomas, are diverse and are characterized by difficulty in pathological diagnosis. In fact, after re-examining patients who had been diagnosed with olfactory neuroblastoma in other hospitals, they may sometimes experience benign diseases such as pituitary adenoma and vice versa.
The reason for such a difficult diagnosis is that the frequency of the disease itself is very low, so there is little experience on the medical side, the pathology (cell tissue) cannot be collected in large amounts, and the cell tissue is often destroyed during processing. May destroy internal tissues), making accurate diagnosis difficult.
The rapid progress in the technology of pathological diagnosis and the ever-changing diagnosis methods and disease classifications themselves make the diagnosis difficult. It is important to consult a physician experienced in diagnosis for this disease.(3)
Location And Symptoms Of Olfactory Neuroblastoma
Most olfactory neuroblastomas occur in the nasal cavity and ethmoid sinuses. Although it is initially asymptomatic, the main symptoms include nasal congestion. However, patients with such conditions are unlikely to trigger a visit to the hospital, and after a few months when other symptoms start appearing (nose bleeding), then only they are referred for diagnosis.
When To Go To Your Doctor For Olfactory Neuroblastoma?
Once you experience recurring symptoms of the disease, you should consult a doctor. It is better to visit an ENT specialist for a proper diagnosis. However, it may be problematic to have a correct diagnosis of olfactory neuroblastoma. Once you are confirmed about the disease the next step is to take important decisions regarding the treatment course.
Olfactory Neuroblastoma 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 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 an increased dose of radiation at one time, new radiotherapy such as particle beam irradiation, and multidisciplinary treatment using different chemotherapy is performed.
Other treatment procedures focus on symptomatic relief depending on the stage of cancer. Palliative care may be advised for end-stage cancer patients suffering from olfactory neuroblastoma.(4)
Survival And Course Of Olfactory Neuroblastoma
According to statistics to date, the 5-year survival rate after treatment for olfactory neuroblastoma, including stage 4, is about 60%. Olfactory neuroblastoma requires long-term follow-up. Relapses are most common in the first few years, but some patients relapse after 10 to 20 years following treatment. Endoscopic intranasal sinus surgery for olfactory neuroblastoma is still in its early days. So, it is necessary to follow the course strictly for the next 10 to 20 years.
- 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.
- 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.
- 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.
- Wu J-Y, Chiou J-F, Ting L-L. Chemo-radiotherapy for olfactory neuroblastoma: cases report and literature review. 2019.
- What is Esthesioneuroblastoma or Olfactory Neuroblastoma: Causes, Symptoms, Treatment, Recovery, Prognosis, Staging
- Is Olfactory Neuroblastoma Hereditary?
- Can Olfactory Neuroblastoma Go Away On Its Own & What Are Its Natural Remedies?
- Is Olfactory Neuroblastoma A Serious Condition & Can It Be Reversed
- Is Olfactory Neuroblastoma Hereditary & Is It A Disability?
- What Is The Prognosis For Olfactory Neuroblastoma & Lifestyle Changes For It?
- What To Eat & Avoid When You Have Olfactory Neuroblastoma?