Olfactory Neuroblastoma

Olfactory Neuroblastoma

Summarize

Olfactory neuroblastoma is a small round cell tumor that originates in the nasal nerve epithelium. The incidence of olfactory neuroblastoma in children under 15 years of age is estimated to be one in a million, making it a very rare malignant tumor in children.

        Although extremely rare, neuroblastoma of the olfactory nerve is the most common type of nasal cancer in pediatric patients.

Epidemiological

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Etiology & Risk Factors

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Classification & Staging

Staging of pediatric olfactory neuroblastoma  cell

        Olfactory neuroblastoma can be staged according to the Kadish system (refer to Table 1). Survival rates range from 90% in stage A to less than 40% in stage D. Most patients have locally advanced disease (Kadish stages B and C), and nearly one-third of patients have metastatic tumors (Kadish stage D). Recent studies suggest that PET-CT may aid in staging this disease.

Table 1. Kadish staging system

Clinical Manifestations

 clinlcal symptom

        Most children with the disease develop the following symptoms between the ages of 10 and 20:

50.  rhinobyon ​
50.  hemorrhinia
50.  hypopiesia
50.  exophthalmos
50. The nasopharyngeal mass may extend locally to the orbit, paranasal sinuses or frontal lobe.

Clinical Department

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Examination & Diagnosis

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Clinical Management

Treatment and prognosis of childhood olfactory neuroblastoma

        Thanks to the use of multimodal therapy to optimize survival, children have a 5-year survival rate of more than 70 percent after their initial diagnosis.

        Depending on the stage of the Kadish, the treatment program includes:

Kadish A stage: if the surgical margin is negative, surgery alone is performed. Adjuvant radiotherapy is suitable for patients with a positive margin or residual lesions. ​● Kadish B stage: adjuvant radiotherapy is performed after surgery. The role of adjuvant chemotherapy is still controversial.

Kadish C stage: chemotherapy, radiotherapy or synchronous chemotherapy and radiotherapy followed by surgery.

Kadish D phase: systemic chemotherapy and radiotherapy for local and metastatic tumors.

        The current mainstream treatments are surgery and radiotherapy. Newer techniques, such as endoscopic sinus surgery, can provide similar short-term outcomes to traditional open craniectomy. Other techniques, such as stereotactic radiosurgery and proton therapy, may also play a role in the treatment of this tumor.

Prognosis

Prognostic factors

        Based on a retrospective study of mainly adult patients, the following factors may be associated with poor prognosis:

50. Higher histopathological grade ​
50. The surgical margins were positive for pathology.
50.  cervical lymph nody metastasis

Follow-up & Review

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Daily Care

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Cutting-edge therapeutic and clinical Trials

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References

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Audit specialists

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