Aesthesioneuroblastoma is a rare tumor inside the nose. Esthesioneuroblastoma is also known as olfactory neuroblastoma and is one of the cancers of the nasal mucosa. Doctors estimate the frequency of esthesioneuroblastoma to be less than 1:1,000,000.
What is esthesioneuroblastoma?
A first scientific description of the esthesioneuroblastoma was made in 1924 by the doctors Luc and Berger. Esthesioneuroblastoma develops from the olfactory mucosa and in particular from the neuroepithelium. The olfactory mucosa is mainly located on the so-called lamina cribrosa. For dislocation definition and meaning, please visit howsmb.com.
Sometimes the esthesioneuroblastoma also forms on the septum of the nose or in the upper area of the turbinates. Basically, esthesioneuroblastomas are extremely rare in the population, with researchers assuming a prevalence of less than 1:1,000,000. It is unknown whether esthesioneuroblastoma occurs more frequently in men or women. New findings indicate that the malignant tumor arises from a stem cell of the olfactory mucosa as a result of degeneration.
Other diseases such as sarcomas, carcinomas or lymphomas also occur in the same section of the nose. However, esthesioneuroblastoma can be differentiated from these diseases by simple microscopic examinations. Esthesioneuroblastoma develops in different stages, which are characterized by increasing severity.
In the early stages, esthesioneuroblastoma grows inside the nasal cavity. In the advanced stage of the disease, the tumor extends from the nasal cavity into at least one paranasal sinus. In the final stages of esthesioneuroblastoma, the tumor also spreads outside the paranasal sinuses, for example into the base of the skull, the brain or the lymph nodes in the neck. In addition, distant metastases sometimes develop.
The exact causes of esthesioneuroblastoma have not yet been clarified. Modern medical research studies indicate that esthesioneuroblastoma may develop from an olfactory stem cell after that cell has degenerated. In principle, there is still a need for research into the pathogenesis of esthesioneuroblastoma, as with numerous other types of tumors.
Symptoms, Ailments & Signs
The typical symptoms of esthesioneuroblastoma are first noticeable in the area of the nose and in connection with its functions. The nasal passages are blocked by esthesioneuroblastoma and nosebleeds are common. Patients with esthesioneuroblastoma suffer from increased nasal discharge, experience pain and progressively lose their sense of smell.
Basically, the esthesioneuroblastoma starts from the olfactory cells. The tumor occurs more frequently in people between the ages of 20 and 50. Aesthesioneuroblastoma is characterized by slow growth and initially remains mostly localized. As esthesioneuroblastoma progresses, distant metastases sometimes develop in organs such as the lungs, bones, brain and cervical lymph nodes.
Diagnosis & course of disease
The diagnosis of esthesioneuroblastoma is made, for example, by a specialist in ear, nose and throat medicine. In the initial consultation with the patient, the doctor discusses the symptoms and the beginning of the complaints. In the clinical examination of the sick person, the specialist mainly uses imaging methods.
A CT or MRI scan visualizes the esthesioneuroblastoma and shows the size and location of the tumor. In addition, histological analyzes are used in the diagnosis of esthesioneuroblastoma. The diagnosis of the tumor takes place on average six months after the onset of symptoms of esthesioneuroblastoma.
Rapid diagnosis is extremely important for a favorable prognosis of esthesioneuroblastoma. Because then a timely removal of the tumor is possible. It is important to distinguish esthesioneuroblastoma from other malignant diseases of the interior of the nose, such as sarcomas, carcinomas or lymphomas. The differential diagnosis is usually made by means of light microscopic examinations. The esthesioneuroblastoma must also be differentiated from the benign nasal polyposis.
In many cases, esthesioneuroblastoma is treated relatively late, so that metastases have already formed. In this case, a significantly reduced life expectancy for the patient is to be expected. However, if the tumor is diagnosed early, it can be removed.
Those affected often suffer from nosebleeds due to esthesioneuroblastoma, which can occur for no particular reason and without injury. A strong discharge from the nose can also occur and have a very negative effect on the patient’s quality of life. Most sufferers also experience nasal pain and an altered sense of smell.
The nose itself often appears blocked, which can lead to breathing difficulties. Aesthesioneuroblastoma can usually be treated with radiation therapy. There are no complications. Surgery may also be necessary. However, the further course depends strongly on the time of diagnosis of the esthesioneuroblastoma. Because the tumor can come back, most patients need further regular check-ups with a doctor to avoid these complications.
When should you go to the doctor?
Repeated or intermittent nosebleeds are a cause for concern. A doctor’s visit is necessary if there were no external influences that caused the bleeding. If the affected person loses their sense of smell or if they notice limitations in their olfactory perception, a medical examination is advisable. This development can be a disease that requires action. A doctor should also be consulted if there is a discharge from the nose, swelling in the area of the nose and problems with breathing. Snoring, a decrease in physical performance and discomfort when performing sporting activities indicate a health problem.
A doctor should be consulted to clarify the cause, since a diagnosis can only be made using various medical tests. If irregularities or pain are noticed with a cold, a doctor’s visit is necessary immediately. Changes in the complexion, headaches or sleep disorders are further signs of an illness. Since untreated serious health developments and complications can occur, a doctor should be consulted at the first irregularities. A blocked nose is a sign of a tissue change. Since the cancer can lead to premature death in severe cases, treatment is needed at an early stage.
Treatment & Therapy
In the treatment of esthesioneuroblastoma, the focus is on the complete removal of the tumor as part of a surgical intervention. Following the operation, patients usually undergo radiation therapy. In addition, the treatment of esthesioneuroblastoma sometimes takes place in the reverse order.
The cure rate for esthesioneuroblastoma is between 50 and 80 percent. While distant metastases are relatively rare, there is a risk of recurrence at the original site of the esthesioneuroblastoma. In people who have first-time surgery for esthesioneuroblastoma, the disease is considered cured about 50 percent of the time.
The prognosis of esthesioneuroblastoma improves the younger the patient is at the time of the initial disease. In addition, the extension of the esthesioneuroblastoma to the paranasal sinuses and the histological findings affect the prognosis of the disease. As a result of the surgical removal of the esthesioneuroblastoma, patients lose their sense of smell in whole or in part. The medium-term probability of survival for esthesioneuroblastoma is around 70 percent and relates to a time window of two to five years after the diagnosis was made.
If local recurrences of the tumor occur, further therapeutic interventions are necessary. Overall, esthesioneuroblastoma requires continuous medical monitoring even after successful resection and radiotherapy. In this way, doctors can quickly identify possible recurrences of the esthesioneuroblastoma inside the nose so that appropriate countermeasures can be taken.
Esthesioneuroblastoma cannot be effectively prevented because the causes of the tumor are unclear. Prompt diagnosis and treatment improve the prognosis of esthesioneuroblastoma, which is best in young patients and complete resection of the tumor.
You can do that yourself
Depending on the course and severity, an esthesioneuroblastoma can in some cases significantly restrict the patient’s everyday life. Many of those affected suffer from severe insomnia and a stuffy nose. It is therefore often not possible to fulfill a professional activity and other obligations.
Heavy and unexpected bleeding usually occurs. Those affected should therefore always ensure that they always carry handkerchiefs with them. In addition, patients usually experience considerable limitations as a result of the treatment of esthesioneuroblastoma. For this reason, they are often dependent on the help of family members or relatives. During therapy, those affected are usually unable to work. In rare cases, however, the cancer does not cause any symptoms, so that it initially goes completely unnoticed. This sometimes even leads to the premature death of the patient.
Especially during therapy, it is important that those affected eat a healthy and balanced diet. It is advisable to consult a nutritionist for this purpose. In addition, people with esthesioneuroblastoma should stop smoking and consuming alcoholic beverages. Since cancer is usually a heavy burden, it is sometimes advisable to visit a self-help group to exchange ideas with other patients.