Esthesioneuroblastoma (Olfactory Neuroblastoma)
Esthesioneuroblastoma (olfactory neuroblastoma) is a rare malignant neuroectodermal tumor arising from the olfactory epithelium in the upper nasal cavity. It grows slowly but behaves in a locally invasive manner, and can extend through the skull base into the anterior cranial fossa.
Останнє оновлення: 2026-06-06
Definition
Esthesioneuroblastoma, first described in 1924, is a rare malignant tumor arising from the basal neural cells of the olfactory epithelium at the roof of the nasal cavity. It accounts for about 3-6% of all sinonasal tumors. It begins adjacent to the cribriform plate and may grow slowly to involve the anterior skull base and, rarely, the frontal lobe.
Epidemiology and Risk Factors
The disease shows a bimodal age distribution, with a first peak at 11-20 years and a second at 50-60 years. The sex distribution is roughly equal or slightly male-predominant. Incidence is about 0.4 cases per million per year. Possible risk factors include chronic sinonasal inflammation, smoking and certain occupational exposures (wood dust, metal dust); most cases are sporadic.
Symptoms
The most common presenting complaint is progressive unilateral nasal obstruction. This may be accompanied by recurrent nosebleeds (epistaxis), loss or reduction of smell (anosmia/hyposmia) and frontal headache. Orbital involvement can cause proptosis and double vision. Intracranial extension may produce olfactory hallucinations, personality changes and seizures.
Diagnosis and Staging
Contrast-enhanced paranasal sinus and brain MRI is the gold standard, showing cribriform plate involvement and intracranial extension. Paranasal CT better demonstrates bony destruction. PET-CT is used for staging and metastasis screening. Nasoendoscopy reveals the mass and allows biopsy, which should follow imaging. Definitive diagnosis is confirmed by immunohistochemistry (chromogranin, synaptophysin, NSE positive). The Kadish system (A-D) is used for staging and the Hyams histologic grade (1-4) for prognosis.
Treatment
Treatment is multimodal and the best results are achieved with surgery. For early-to-intermediate stage disease, endoscopic endonasal surgery has become the leading minimally invasive approach; it requires no external incision, allows faster recovery and can be applied even in selected advanced cases. Open craniofacial resection may be required for extensive frontal lobe invasion or wide dural involvement. Adjuvant radiotherapy after surgery is standard and improves local control; proton therapy offers normal-tissue sparing in intracranial extension and in younger patients. Chemotherapy (usually cisplatin-based) is added in high-grade or advanced disease.
Prognosis and Follow-up
Prognosis depends on stage and histologic grade. In general, 5-year survival is high in early-stage (Kadish A-B) disease and lower in advanced-stage (Kadish C-D) disease; low-grade (Hyams 1-2) tumors follow a better course. Because the disease can recur late (10-20 years later), lifelong follow-up is needed, with regular MRI and nasoendoscopy, more frequent in the early years. Outcomes are individual and none can be guaranteed.
Джерела
- Greenberg MS. Greenberg's Handbook of Neurosurgery. 10th ed. Thieme; 2023:951.
- Osborn AG, Hedlund GL, Salzman KL. Osborn's Brain: Imaging, Pathology, and Anatomy. 2nd ed. Elsevier; 2018:651.
- Dulguerov P, et al. Esthesioneuroblastoma: a meta-analysis and review. Lancet Oncol. 2001.
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