# Esthesioneuroblastoma (olfactory neuroblastoma)

Source: https://onco.cc/cancers/esthesioneuroblastoma/  
OnCo record `esthesioneuroblastoma` (Cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

Esthesioneuroblastoma is a rare cancer of the nasal cavity and sinuses that arises from the smell-sensing olfactory nerve lining at the roof of the nose, next to the brain. It is treated with surgery through the nose or skull base followed by radiotherapy, with chemotherapy added for high-grade or widespread tumours, and because it can return a decade or more later patients are followed for life.

## Summary

Esthesioneuroblastoma arises from the olfactory neuroepithelium of the upper nasal vault and cribriform plate, and its position against the anterior skull base means it grows into the orbit and the frontal lobes as readily as into the sinuses. It presents with nasal obstruction, bleeding and loss of smell, and is staged by the Kadish system (A: nasal cavity; B: paranasal sinuses; C: beyond, including orbit and skull base; D: nodal or distant metastases, added later) and graded by Hyams (I to IV) on the degree of differentiation, rosettes, necrosis and mitotic activity; Hyams grade is the strongest predictor of survival, with low-grade tumours behaving indolently and high-grade tumours recurring early and spreading to neck nodes and distant sites. Immunohistochemistry (synaptophysin, chromogranin, S100 sustentacular cells) separates it from sinonasal undifferentiated carcinoma, NUT carcinoma, melanoma and lymphoma, which share the site. Most tumours express somatostatin receptor 2, which allows DOTATATE PET imaging and, in relapse, radioligand therapy; IDH2 mutations, typical of sinonasal undifferentiated carcinoma, occur in a subset of high-grade esthesioneuroblastomas.

Treatment is multimodal. Craniofacial resection through a combined transfacial and transcranial approach was the standard from the 1970s, and expanded endonasal endoscopic resection with skull base reconstruction now achieves equivalent margins with less morbidity in experienced hands; postoperative radiotherapy is recommended for almost all patients because it improves local control, and intensity-modulated or proton therapy spares the optic pathways and brain. Chemotherapy, usually cisplatin with etoposide, is given as induction for Kadish C and D or Hyams III to IV tumours, sometimes with radiotherapy for unresectable disease, and elective neck irradiation is considered for high-grade tumours because late nodal recurrence is common. Recurrence occurs in up to a third of patients, often years or decades later, so surveillance MRI continues indefinitely; relapses are treated with repeat surgery, re-irradiation or radioligand therapy with lutetium-177 dotatate where the tumour takes up the tracer, and platinum-etoposide or temozolomide for metastatic disease. Because of the tumour's rarity, care belongs in a skull base team, and prospective data are limited to registry series.

## Fields

- Kind: Cancer
- Last checked: 2026-09-18
- Also known as: Olfactory neuroblastoma; ONB; Esthesioneuroepithelioma; Neuroblastoma of the olfactory epithelium
- Tags: subtype-page; head-and-neck
- Group: head and neck
- Burden: A rare tumour, a few percent of sinonasal cancers, affecting all ages with peaks in young adults and the middle-aged; slow-growing in its low-grade form, aggressive in its high-grade form, and prone to relapse many years after treatment.
- Subtypes: Low-grade esthesioneuroblastoma (Hyams I to II; indolent); High-grade esthesioneuroblastoma (Hyams III to IV; aggressive, chemotherapy); Kadish A to B esthesioneuroblastoma (nasal cavity and sinuses; surgery and radiotherapy); Kadish C esthesioneuroblastoma (orbit or skull base; multimodal); Kadish D esthesioneuroblastoma (nodal or distant metastases); Recurrent esthesioneuroblastoma (late; somatostatin-receptor-directed therapy considered)
- Biomarkers: Hyams grade (I to IV; strongest prognostic factor); Kadish or modified Kadish stage (A to D); Synaptophysin, chromogranin and S100 sustentacular cell pattern (diagnosis); Somatostatin receptor expression and DOTATATE PET uptake (imaging, radioligand eligibility); IDH2 mutation (subset of high-grade tumours); Neck node status on MRI or PET-CT

## Sections of this record

The page is a hub with ten sections in reading order; large sections have their own page. The same plan as JSON: https://onco.cc/api/v1/cancers/esthesioneuroblastoma/sections.json

- Overview (on the hub): The TL;DR, the family this cancer belongs to, the organ, who gets it and what the state of the art is. https://onco.cc/cancers/esthesioneuroblastoma/#overview [3 state-of-the-art points]
- Types and stages (on the hub): Anatomy, the subtypes and how they differ, how it is staged, and where advanced disease spreads. https://onco.cc/cancers/esthesioneuroblastoma/#what-it-is [6 subtypes]
- Symptoms and diagnosis (on the hub): How this cancer shows itself, how the diagnosis is confirmed, and the biomarkers clinicians test for. https://onco.cc/cancers/esthesioneuroblastoma/#finding-it [6 biomarkers]
- Treatment (on the hub): The standard of care by setting, the medicines, surgery and radiotherapy named in it, and the regimens behind them. https://onco.cc/cancers/esthesioneuroblastoma/#treating-it [5 settings, 4 decisions with options]
- Trials and papers (on the hub): Trials recruiting now, the landmark trials, the trials held by this cancer's subtypes, the key papers and what they mean, the latest literature, and the milestones year by year. https://onco.cc/cancers/esthesioneuroblastoma/#evidence [2 key papers, 6 milestones]
- Biology and targets (on the hub): The molecular landscape: the targets and how often each appears, the pathways, the mechanics stages and the preclinical models. https://onco.cc/cancers/esthesioneuroblastoma/#science [2 targets]
- Countries and centres (own page): Cases by country, the UK and NHS pathway and other country lenses, the expert centres with trials on record, and the centres named on this cancer's subtypes. https://onco.cc/cancers/esthesioneuroblastoma/where-you-are/
- Decisions and support (on the hub): The decisions you may face, the aids that walk through them, the warnings on record, the first sixty days and the questions to ask. https://onco.cc/cancers/esthesioneuroblastoma/#living-with-it [16 questions, 6 red cards]
- Pipeline and open problems (own page): Everything in development, the medicines held by this cancer's subtypes, the open problems and what is being done about them, the roadmaps, and what changed on this record. https://onco.cc/cancers/esthesioneuroblastoma/coming/ [5 medicines, 4 open problems]
- Data (own page): Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked. https://onco.cc/cancers/esthesioneuroblastoma/data/ [31 connected records]

## Standard of care

- Diagnosis and staging: Endoscopic biopsy with immunohistochemistry, MRI and CT of the sinuses and skull base, neck imaging, DOTATATE PET where available. ([Histopathology & immunohistochemistry](https://onco.cc/technologies/histopathology-ihc/), [MRI](https://onco.cc/technologies/mri/), [CT (computed tomography)](https://onco.cc/technologies/ct/), [Somatostatin receptor PET (68Ga/64Cu-DOTATATE)](https://onco.cc/technologies/sstr-pet/), [PET/CT](https://onco.cc/technologies/pet-ct/))
- Resectable disease: Endoscopic endonasal or craniofacial resection with skull base reconstruction by a skull base team, followed by postoperative radiotherapy (intensity-modulated or proton) for nearly all patients. ([Robotic & minimally invasive surgery](https://onco.cc/technologies/robotic-surgery/), [IMRT / IGRT (modern external beam)](https://onco.cc/technologies/imrt-igrt/), [Proton therapy](https://onco.cc/technologies/proton-therapy/))
- High-grade or advanced disease (Hyams III to IV, Kadish C to D): Induction cisplatin and etoposide, then surgery and radiotherapy or definitive chemoradiotherapy; elective neck irradiation considered. ([Cisplatin](https://onco.cc/drugs/cisplatin/), [Etoposide](https://onco.cc/drugs/etoposide/), [Platinum + etoposide (EP / CE)](https://onco.cc/drugs/platinum-etoposide/), [IMRT / IGRT (modern external beam)](https://onco.cc/technologies/imrt-igrt/), [Chemoradiation (chemoradiotherapy, CRT)](https://onco.cc/terms/chemoradiation/))
- Recurrent or metastatic disease: Repeat surgery or re-irradiation for local relapse; lutetium-177 dotatate for somatostatin-receptor-positive disease; platinum-etoposide or temozolomide; trials. ([Re-irradiation](https://onco.cc/terms/re-irradiation/), [Lutetium-177 dotatate](https://onco.cc/drugs/lutathera/), [Peptide receptor radionuclide therapy (PRRT)](https://onco.cc/technologies/prrt/), [Platinum + etoposide (EP / CE)](https://onco.cc/drugs/platinum-etoposide/), [Temozolomide](https://onco.cc/drugs/temozolomide/))
- Follow-up: MRI surveillance indefinitely because of late relapse; management of anosmia, cerebrospinal fluid leak and visual effects. ([MRI](https://onco.cc/technologies/mri/))

## State of the art

- Endoscopic skull base surgery has replaced open craniofacial resection for most tumours with less morbidity.
- Hyams grade guides who needs chemotherapy.
- Somatostatin receptor imaging and radioligand therapy offer a targeted option in relapse.

## Open problems

- No prospective trial has ever been conducted; every recommendation rests on series.
- Late relapse after ten or more years makes cure hard to define.
- The role and timing of chemotherapy in intermediate-grade tumours is unclear.
- Elective neck treatment is debated.

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Esthesioneuroblastoma
- NCCN Head and Neck Cancers (ethmoid sinus): https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1437
- Wikipedia: https://en.wikipedia.org/wiki/Esthesioneuroblastoma

## Connected records

- cancers: [Nasal cavity and paranasal sinus cancers (including esthesioneuroblastoma)](https://onco.cc/cancers/sinonasal/), [Neuroblastoma (paediatric)](https://onco.cc/cancers/neuroblastoma/), [NUT carcinoma (midline carcinoma with NUTM1 rearrangement)](https://onco.cc/cancers/nut-carcinoma/), [Sinonasal undifferentiated carcinoma (SNUC) and SWI/SNF-deficient sinonasal carcinoma](https://onco.cc/cancers/sinonasal-undifferentiated-carcinoma/)
- technologies: [CT (computed tomography)](https://onco.cc/technologies/ct/), [Histopathology & immunohistochemistry](https://onco.cc/technologies/histopathology-ihc/), [IMRT / IGRT (modern external beam)](https://onco.cc/technologies/imrt-igrt/), [MRI](https://onco.cc/technologies/mri/), [Peptide receptor radionuclide therapy (PRRT)](https://onco.cc/technologies/prrt/), [PET/CT](https://onco.cc/technologies/pet-ct/), [Proton therapy](https://onco.cc/technologies/proton-therapy/), [Radioligand therapy (beta emitters)](https://onco.cc/technologies/radioligand-therapy/), [Robotic & minimally invasive surgery](https://onco.cc/technologies/robotic-surgery/), [Somatostatin receptor PET (68Ga/64Cu-DOTATATE)](https://onco.cc/technologies/sstr-pet/)
- targets: [Somatostatin receptor 2](https://onco.cc/targets/sstr2/)
- drugs: [Cisplatin](https://onco.cc/drugs/cisplatin/), [Etoposide](https://onco.cc/drugs/etoposide/), [Lutetium-177 dotatate](https://onco.cc/drugs/lutathera/), [Platinum + etoposide (EP / CE)](https://onco.cc/drugs/platinum-etoposide/), [Temozolomide](https://onco.cc/drugs/temozolomide/)
- terms: [Chemoradiation (chemoradiotherapy, CRT)](https://onco.cc/terms/chemoradiation/), [Disease-specific staging and risk systems (FIGO, Ann Arbor, IPI, R-ISS, ELN, IMDC)](https://onco.cc/terms/staging-systems/), [Head and neck subsites (oral cavity, oropharynx, larynx)](https://onco.cc/terms/head-neck-subsites/), [Rare cancers](https://onco.cc/terms/rare-cancers/), [Re-irradiation](https://onco.cc/terms/re-irradiation/)
- key papers: [Esthesioneuroblastoma: a meta-analysis and review](https://onco.cc/key-papers/paper-dulguerov-esthesioneuroblastoma-meta-analysis-lancet-oncol-2001/), [Kadish staging: olfactory neuroblastoma, a clinical analysis of 17 cases](https://onco.cc/key-papers/paper-kadish-olfactory-neuroblastoma-staging-cancer-1976/)

---
JSON: https://onco.cc/api/v1/entities/esthesioneuroblastoma.json