Health condition · Clinically reviewed
Esthesioneuroblastoma, endoscopic skull base surgery, radiotherapy and proton beam therapy.
A rare malignant tumour of the olfactory epithelium at the top of the nose. Treated in a small number of UK skull base centres, with a very specific pathway from biopsy to MDT plan.
Why trust this guide
- 01
Skull base specialist reviewed
Written by our editorial team and reviewed against UK skull base centre practice before publication.
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Sourced from guidance
Checked against NHS England commissioned skull base pathways, ENT UK and peer-reviewed sinonasal oncology literature.
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Current for 2026
Reflects modern practice including endoscopic endonasal resection, proton beam therapy and MDT-led adjuvant care.
Key facts
Esthesioneuroblastoma at a glance.
The essentials, in plain English - what it is, how it is staged and how it is treated in the UK today.
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What it is
A rare malignant neuroectodermal tumour arising from the olfactory epithelium at the top of the nasal cavity and cribriform plate.
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How common
Roughly 3% of sinonasal malignancies. UK incidence is very low - a few dozen new cases each year.
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Who it affects
Bimodal age distribution with peaks at 15 to 20 and 50 to 60. Men and women affected equally.
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Staging
Kadish stages A to D describe local extent, with Hyams histological grades I to IV describing tumour biology.
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Mainstay treatment
Endoscopic endonasal skull base resection with cribriform plate and dural resection, followed by radiotherapy in most cases.
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Where it is treated
Only in NHS England commissioned skull base centres such as UCLH, the Royal Marsden, Manchester, Bristol, Birmingham and Newcastle.
Why this guide matters
Rare, but with a very specific pathway.
Because esthesioneuroblastoma is uncommon it is easy to miss - and it must be treated at a commissioned skull base centre. Three anchors shape the rest of this page.
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Unilateral symptoms are the clue
Persistent one sided nasal obstruction, anosmia or epistaxis in an adult should always be escalated to ENT - not repeatedly treated as sinusitis.
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A specialist MDT sets the plan
Kadish stage, Hyams grade, imaging and pathology are reviewed together by ENT, neurosurgery, medical and clinical oncology at a UK skull base centre.
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Modern surgery changed outcomes
Endoscopic endonasal resection with cribriform plate removal, combined where needed with radiotherapy or proton beam therapy, is now the mainstay.
How the diagnosis is made
From nasoendoscopy to a specialist MDT plan.
The steps a UK ENT team and skull base MDT will normally follow, in order - so you know what to expect and why each step matters.
Phase 1 · Assessing
Endoscopy, imaging and biopsy
Phase 2 · Confirming
Pathology, staging and grading
Phase 3 · Planning
Skull base MDT plan
- 01
Assessing
Flexible nasoendoscopy
A thin flexible camera through the nose lets an ENT surgeon see a mass high in the nasal cavity that a standard exam would miss.
- 02
Assessing
Cross sectional imaging
MRI plus CT of the sinuses and head to map the tumour, the cribriform plate, the orbit and any intracranial extension.
- 03
Assessing
Specialist ENT biopsy
Tissue is taken by an ENT surgeon with skull base experience so the sample is representative and safe.
- 04
Confirming
Head and neck pathology review
A specialist pathologist confirms the diagnosis with immunohistochemistry - S100, synaptophysin, chromogranin, NSE and neurofilament.
- 05
Confirming
Hyams grade and Kadish stage
The pathologist assigns a Hyams grade (I to IV) and the team assigns a Kadish stage (A to D) to guide treatment intensity.
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Confirming
PET-CT staging
Whole body PET-CT looks for cervical nodal disease and rare distant spread that would change the plan.
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Planning
Skull base MDT
The full plan is set at a specialist MDT with ENT, neurosurgery, medical oncology and clinical oncology in the room together.
Typical timeline: from ENT referral to MDT plan within a matter of weeks at a commissioned centre.
Symptoms
What esthesioneuroblastoma actually feels like.
The classic mix of unilateral nasal, sinus and neurological symptoms - and the features that mean it is time to escalate to a specialist.
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Unilateral nasal obstruction
A blocked nose on one side that does not clear with decongestants is the most common early symptom.
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Anosmia
Loss of smell reflects damage to the olfactory epithelium where the tumour originates - often an early clue.
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Recurrent epistaxis
Repeated nosebleeds from one side, particularly in an adult, should prompt ENT referral rather than reassurance.
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Facial pain or pressure
A dull ache over the cheek, forehead or between the eyes can reflect tumour extending into the paranasal sinuses.
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Persistent rhinorrhoea
Clear or blood tinged discharge from one side of the nose that will not settle - a red flag in adult ENT practice.
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Orbital symptoms
A bulging eye, double vision or reduced vision points to orbital invasion and needs urgent skull base review.
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Neurological features
Headache, personality change or seizures can occur when the tumour extends through the cribriform plate into the frontal lobe.
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Red flag - unilateral persistent
Any adult with unilateral nasal symptoms lasting more than a few weeks deserves urgent ENT assessment - not another decongestant course.
Treatment
How esthesioneuroblastoma is treated in the UK.
Endoscopic skull base surgery first, with radiotherapy for most patients, proton beam therapy for selected cases and chemotherapy in higher risk disease.
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Endoscopic endonasal resection
The modern mainstay - specialist skull base surgeons remove the tumour, cribriform plate and dura through the nose. See our page on endoscopic skull base surgery.
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Combined transnasal transcranial
For extensive intracranial disease a joint ENT and neurosurgical approach through the nose and skull gives a safer resection with clear margins.
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Adjuvant radiotherapy
Post-operative radiotherapy at 60 to 70 Gy to the tumour bed is standard in most cases, protecting eyes, optic nerves and brain.
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Proton beam therapy
Selected patients are referred for proton beam therapy to reduce dose to the frontal lobes, eyes and optic pathways. See our proton beam therapy page.
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Platinum based chemotherapy
Cisplatin and etoposide are used in higher grade or advanced disease, either before surgery (neoadjuvant) or after (adjuvant), always at a specialist centre.
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Selective neck dissection
When there is confirmed or high risk cervical nodal disease, a selective neck dissection removes the involved node levels.
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Elective neck irradiation
For higher stage or grade tumours the neck may be irradiated electively to reduce the risk of later nodal relapse.
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Emerging targeted therapy
Molecular profiling is starting to identify targetable pathways in rare sinonasal cancers - options are still emerging and usually within a clinical trial.
What this guide is based on
The sources behind every claim on this page.
NHS England commissioning guidance, specialist society standards and peer reviewed sinonasal oncology literature current at the time of last review.
Key references
Guidelines and standards we relied on.
A quiet reminder
This guide is for information, not medical advice.
Your specialist MDT knows the details of your scans, pathology and history and can tell you which parts apply to you. If in doubt, get in touch with your clinical nurse specialist.
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NHS England. Specialised services for skull base surgery and complex head and neck cancer.
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ENT UK. Position statements on sinonasal and skull base malignancy.
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European Society for Medical Oncology (ESMO). Clinical practice guidelines for nasal cavity and paranasal sinus cancers.
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Royal College of Radiologists. Guidance on radiotherapy and proton beam therapy for skull base tumours.
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Cancer Research UK. Information for patients on rare head and neck cancers.
Red flags
When symptoms need urgent attention.
Most sinonasal symptoms are benign. These are the situations that are not - where a specialist opinion or emergency care is needed.
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Sudden visual loss
A rapid change in vision, a bulging eye or new double vision needs same day skull base review - not a routine appointment.
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New neurological symptoms
Personality change, seizures, weakness or severe headache can signal intracranial extension and needs urgent imaging.
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Heavy or repeated epistaxis
Nosebleeds that are heavy, repeated or one sided in an adult should always prompt ENT assessment rather than watchful waiting.
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CSF leak
Clear watery fluid dripping from one nostril, especially after biopsy or surgery, may be cerebrospinal fluid and needs urgent review.
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Rapid facial swelling
A quickly enlarging facial or periorbital mass suggests aggressive local extension and warrants same week specialist input.
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Post-treatment relapse signs
New unilateral symptoms after treatment - obstruction, bleeding, anosmia or pain - need prompt skull base follow-up.
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Neck lump
A firm lump in the neck after diagnosis raises the possibility of cervical nodal spread and needs urgent MDT review.
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Meningitis features
Fever, neck stiffness and headache after skull base surgery is a medical emergency and needs A&E attendance.
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Psychological distress
A rare cancer diagnosis carries a heavy mental health burden - Macmillan and clinical psychology support should be offered early.
Living with it
A rare cancer, treated by a specialist team.
Four things that make the biggest difference day to day - staying connected to your MDT, adjusting to changes in smell and taste, using proper support and speaking up early.
A quiet reminder
Follow-up matters as much as first treatment.
Late recurrence can happen years after treatment, so scheduled scans and specialist review are how outcomes are protected long term.
- 01 Team
Stay linked to your MDT
Follow-up at a commissioned skull base centre is not optional - it is where recurrence is caught early and rehabilitation is coordinated.
- 02 Senses
Adjusting to anosmia
Loss of smell affects taste, appetite and safety. Smoke alarms, gas detectors and dietitian input all help you live well after treatment.
- 03 Support
Use Macmillan and CRUK
Macmillan nurses and Cancer Research UK provide practical, financial and emotional support tailored to rare cancers - lean on them.
- 04 Voice
Speak up between appointments
New symptoms, low mood or side effects should be reported early through your clinical nurse specialist - do not wait for the next scan.
Frequently asked
Everything we get asked about esthesioneuroblastoma.
Quick answers on staging, surgery, radiotherapy, proton beam therapy and follow-up.
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What is esthesioneuroblastoma?
It is a rare malignant neuroectodermal tumour, also called olfactory neuroblastoma, that arises from the olfactory epithelium at the top of the nasal cavity and around the cribriform plate. It makes up about 3% of sinonasal cancers and is treated at specialist skull base centres.
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How is it staged and graded?
The Kadish system stages local extent - A confined to the nasal cavity, B extending into the paranasal sinuses, C beyond those areas, and D with regional nodal or distant spread. Hyams grades I to IV describe how aggressive the tumour looks under the microscope. Together they guide the treatment plan.
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What is the main treatment?
The mainstay is endoscopic endonasal skull base surgery - specialist surgeons remove the tumour together with the cribriform plate and overlying dura. Most people then receive adjuvant radiotherapy at 60 to 70 Gy, and selected patients are considered for proton beam therapy or platinum based chemotherapy.
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Where in the UK is it treated?
Only at NHS England commissioned skull base centres. These include UCLH, the Royal Marsden, Manchester, Bristol, Birmingham and Newcastle among others. Care is coordinated through a specialist MDT of ENT, neurosurgery, medical oncology and clinical oncology.
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Why is proton beam therapy considered?
The tumour sits close to the eyes, optic nerves and frontal lobes. Proton beam therapy delivers a sharper dose fall-off than standard radiotherapy, which can reduce dose to these critical structures. It is offered selectively through the NHS national proton service.
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What is the outlook after treatment?
Outcomes depend heavily on Kadish stage, Hyams grade and completeness of surgery. Long-term survival with modern skull base surgery and radiotherapy is meaningfully better than with older techniques, but recurrence can occur years later, which is why lifelong specialist follow-up matters.
Related content
Keep reading.
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Ewing sarcoma
Another neuroectodermal malignancy.
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Embryonal tumour
Related paediatric neuroectodermal tumour.
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Epithelioid sarcoma
Rare soft tissue malignancy.
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Head and neck cancer
Overview of head and neck malignancy.
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Head and neck cancers
Wider group of related conditions.
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Endoscopic skull base surgery
The mainstay operation for this tumour.
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Proton beam therapy
Precision radiotherapy for selected patients.
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Tumour molecular profiling
Emerging genomic testing for rare cancers.
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Plastic surgery reconstruction
Reconstruction after skull base resection.
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Private MRI scan
Detailed imaging of the skull base.
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Private CT scan
Bony detail of sinuses and cribriform plate.
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Whole exome sequencing
Broad genomic profiling for rare tumours.
Learn more