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Esthesioneuroblastoma (Olfactory Neuroblastoma)

Rare malignant neuroectodermal tumor arising from the olfactory neuroepithelium of the cribriform plate region, presenting with chronic unilateral nasal obstruction and epistaxis, treated with multimodality combined endoscopic-craniofacial resection followed by radiotherapy.

Written by: Saygı Hospital Health Guide Editorial Board
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This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

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What is Esthesioneuroblastoma (Olfactory Neuroblastoma)?

Esthesioneuroblastoma (ENB) is a malignant neuroectodermal neoplasm arising from the olfactory epithelium of the upper third of the nasal cavity, especially the cribriform plate, superior nasal septum, and superior turbinate. It is also called olfactory neuroblastoma. ENB accounts for 3-6% of sinonasal malignancies and 0.3% of all upper aerodigestive tract malignancies.

Age distribution is bimodal, with peaks in the second and sixth decades and slight male predominance. Histology shows small round blue cells with neuroendocrine features; Hyams histologic grading (I-IV) reflects differentiation, mitotic rate, and necrosis and correlates with prognosis. Distant metastasis occurs to cervical lymph nodes (20-30%), bone, lung, or liver; intracranial extension and orbital invasion are common at presentation.

Diagnosis combines nasal endoscopy with biopsy, CT (sclerotic bony changes), and MRI (dumbbell-shaped tumor with intracranial and intranasal components, marginal cysts characteristic). Kadish staging (A: nasal cavity only, B: paranasal sinuses, C: beyond, D: distant metastasis) guides management. Treatment of choice is combined endoscopic and craniofacial (or pure endoscopic) en bloc resection of the cribriform plate, dura, and tumor with negative margins, followed by adjuvant intensity-modulated radiotherapy (60-70 Gy). Chemotherapy is added for high-grade or Kadish C/D disease. Five-year overall survival is 60-80%; long-term endoscopic and MRI surveillance is mandatory for 10+ years.

Symptoms

Chronic unilateral nasal obstruction
Recurrent epistaxis (mild to moderate)
Anosmia or hyposmia (loss of smell)
Unilateral nasal discharge
Headache, frontal pressure
Proptosis, vision changes (orbital extension)
Cervical lymph node enlargement (20-30% nodal metastasis at diagnosis)

Risk Factors

No clear environmental or genetic risk factors established
Bimodal age distribution (10-20 and 50-60 years)
Slight male predominance (1.2-1.5:1)
Possible association with chronic nasal inflammation (unproven)
No clear hereditary syndromes identified
No tobacco or alcohol association unlike other sinonasal cancers
Sporadic occurrence (no familial clusters)

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Persistent unilateral nasal obstruction with anosmia
  • Recurrent unilateral epistaxis with unilateral nasal mass
  • New onset visual changes or proptosis with nasal symptoms
  • Cervical lymphadenopathy with chronic sinonasal symptoms
  • Unilateral sinonasal mass identified on imaging
  • Cranial neuropathies (V, VI) with nasal complaints
  • Persistent unilateral nasal discharge unresponsive to therapy

Treatment Methods

01
Combined endoscopic-craniofacial en bloc resection (cribriform plate, dura, tumor)
02
Pure endoscopic resection for selected early-stage tumors with negative margins
03
Adjuvant intensity-modulated radiotherapy (IMRT) 60-70 Gy
04
Adjuvant chemotherapy (cisplatin/etoposide) for high-grade or Kadish C/D disease
05
Elective neck dissection or neck irradiation for high-grade or N+ disease
06
Long-term endoscopic and MRI surveillance every 3-6 months for 5+ years
07
Multidisciplinary team (skull base ENT, neurosurgery, radiation oncology, medical oncology)

Which Department to Visit?

You can visit our KBB (Kulak Burun Boğaz) department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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