Who Is At Risk For Olfactory Neuroblastoma & Is There A Blood Test For It?

Olfactory neuroblastoma is a rare tumor that originates in the upper olfactory epithelium in the nose. It accounts for 2% to 3% of nasal sinus tumors and affects 0.4 people per 1 million. It can occur in a wide range of ages, from young to elderly, but mostly affects people in their 20s and 60s. There is no gender or racial difference in the onset.(1)

Who Is At Risk For Olfactory Neuroblastoma?

Who Is At Risk For Olfactory Neuroblastoma?

Most cancers are caused by many risk factors, but sometimes neuroblastoma occurs in children who do not have any of the risk factors described below. Neuroblastoma is the most common solid tumor outside the brain in children. It represents 8 to 10% of all childhood cancers.

About 80% of neuroblastomas appear in children under 5 years of age, with an average age of 2.5 years at the time of diagnosis. It is the most common cancer in babies under 1 year of age. Neuroblastoma is very rare in children over 10 years of age. It affects boys a little more than girls.

The common risk factors are:

  • Family history
  • Certain genetic disorders

Family History: About 1-2% of children diagnosed with neuroblastoma have a family history of the disease. The risk of this cancer appears to be highest in siblings or an identical twin of children who already have the disease. Usually, these children suffer from anaplastic lymphoma kinase gene mutation.(4)

Certain Genetic Disorders: Children with few genetic disorders have a higher risk of developing neuroblastoma. If your child has a genetic disorder that increases their risk of developing Olfactory neuroblastoma, they may need to see the doctor more often to check for the condition. The doctor will tell you what tests your child should have and how often.

Hirschsprung’s disease is a disorder characterized by the absence of nerves in parts of the gut, resulting in a malfunction of the large intestine and possible blockage. The syndrome of congenital central hypoventilation is also called primary alveolar hypoventilation. It is a rare disorder that affects breathing. People with this disorder have shallow breathing, especially when they are sleeping.

The neurofibromatosis type 1 (von Recklinghausen disease) affects the development and growth of nerve cells and causes tumors in the nerves and skin. Children with type 1 neurofibromatosis are at higher risk for certain cancers, including neuroblastoma.

The syndrome Beckwith-Wiedemann affects the growth of various body parts. Children with this syndrome are much larger than normal at birth. During their childhood, they grow and gain weight at an unusual rate. These children have a higher chance of acquiring neuroblastoma.

Li-Fraumeni syndrome increases significantly the risk of developing several types of childhood cancer, including neuroblastoma.

The syndrome Costello affects many parts of the body. Children with Costello syndrome often have stunted growth, intellectual disability, a characteristic facial appearance, extra folds of loose skin, and abnormally flexible joints. These children are more likely to have certain types of cancer, including neuroblastoma.(5)

It is a relatively slow-growing tumor that is initially asymptomatic but often develops from nasal congestion and epistaxis. As the tumor grows, olfactory disturbances, headaches, and impaired eye movements may occur.(2)

Is There A Blood Test For Olfactory Neuroblastoma?

The tissue is removed from the tumor and pathologically diagnosed. It is important to understand the extent of the disease by imaging tests such as fiberscope and CT scans along with ultrasound examination, X-ray examination, MRI, etc.

Blood Test: The purpose of blood test is to determine whether high levels of nerve-specific enolase (NSE, blood tumor marker), lactate dehydrogenase (LDH, an enzyme that works when sugar is converted into energy in cells), etc., in the blood, are present. There is no specific blood test for diagnosis.

Urinalysis: Previously, urine tests (group screening tests) were performed on 6-month-old babies. Tests show that catecholamines, which are produced when neuroblastoma (neuroblastoma) tumor cells are formed, are metabolized to vanilmandelic acid and homovanillic acid and excreted in the urine.(3)

Treatment: Olfactory neuroblastoma treatment is based on postoperative radiation therapy in addition to complete surgical resection. In surgery, the tumor is located in the uppermost part of the nose (the base of the skull), so a craniofacial resection that requires cerebral surgical craniotomy may be selected. In recent years, less invasive surgery using a nasal endoscope may be performed for early lesions. Although it is a highly invasive tumor, radiation therapy after surgery reduces the risk of recurrence.

Medication for olfactory neuroblastoma is the mainstay of treatment for unresectable cases and distant metastases. Although there is no established treatment at this time, studies have shown that cisplatin-based pharmacotherapy or cyclophosphamide or vincristine-based pharmacotherapy can give better results. Local therapy may include radiation therapy or surgery after drug therapy.

After treatment, regular follow-up is required. Relapses are most common in the first few years, but it can relapse even after 10 years as well.

References:

  1. 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.
  2. Attwood JE, Jeyaretna DS, Sheerin F, Shah KA. Mixed Olfactory Neuroblastoma and Adenocarcinoma with In Situ Neuroendocrine Hyperplasia. Head and neck pathology. 2019:1-7.
  3. Jiang Y, Mao X, Zhao Y, Fan C. Intracranial immature teratoma invading the nasal cavity mimicking olfactory neuroblastoma: A case report. Medicine. 2018;97(28).
  4. 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.
  5. 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.

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