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Updated: Mar 1, 2026
Identification of OTX1 and OTX2 As Two Possible Molecular Markers for Sinonasal Carcinomas and Olfactory Neuroblastomas
Published on: February 28, 2019
Olfactory neuroblastoma: a single-center experience
Marton König1,2, Terje Osnes3,4, Peter Jebsen5
1Department of Neurosurgery, Oslo University Hospital-Rikshospitalet, PB4950 Nydalen, 0424, Oslo, Norway. m.s.s.konig@medisin.uio.no.
Abstract:
Olfactory neuroblastoma (ONB) is a potentially curable disease, despite being an aggressive malignancy with a poor natural history. Our goal was to evaluate management outcomes for patients with ONB treated at our institution. Our prospective database for brain tumors and the pathology registry of head and neck cancers at Oslo University Hospital were searched to identify all patients treated for ONB between 1998 and 2016. Variables extracted from these databases, supplemented by retrospective chart reviews, underwent thorough analysis. All cases were formally re-examined by a dedicated head and neck pathologist. Twenty patients were identified. Follow-up was 100%. Mean follow-up was 81.5 months for the entire cohort and 120.3 months for patients with no evidence of disease. Fourteen patients underwent treatment of choice including craniofacial resection (CFR) with or without radiotherapy (XRT). Six patients could only receive less extensive treatment; three patients underwent lateral rhinotomy (LR) with or without XRT after being deemed medically unsuitable for CFR, while another three patients received only supportive, non-surgical treatment (due to positive lymph node status in two and to extensive tumor size in one case). Overall and disease-specific survival rates were 100% after 10 years of follow-up when negative surgical margins were achieved by CFR. Positive margins were associated with poorer outcome with no patients surviving longer than 44 months. Long-term survival was also achieved in two cases among patients not eligible for CFR: one case after radical LR and one case after radio-chemotherapy. Advanced disease at presentation (tumor size ≥40 mm, Kadish grades C and D, or TNM IVa and IVb) and positive surgical margins were correlated to significantly dismal survival. Our study suggests that CFR with or without adjuvant XRT is safe and leads to excellent long-time overall and disease-specific survival. Negative surgical margins, tumor size <40 mm, Kadish stage A/B, and TNM stages I-III are independent prognostic predictors of outcome.
Insights
Craniofacial resection (CFR) with or without radiotherapy (XRT) offers excellent survival for olfactory neuroblastoma (ONB) patients when negative margins are achieved. Advanced disease and positive margins correlate with poor outcomes.
Area of Science:
- Oncology
- Head and Neck Surgery
- Pathology
Background:
- Olfactory neuroblastoma (ONB) is an aggressive malignancy with a poor prognosis.
- Despite its aggressive nature, ONB is potentially curable with appropriate management.
- Evaluating treatment outcomes is crucial for optimizing patient care.
Purpose of the Study:
- To assess the management outcomes for patients with olfactory neuroblastoma (ONB).
- To identify prognostic factors influencing survival in ONB patients.
- To evaluate the safety and efficacy of craniofacial resection (CFR) in ONB treatment.
Main Methods:
- A retrospective analysis of 20 ONB patients treated between 1998 and 2016.
- Data collected from prospective brain tumor databases and head and neck cancer registries.
- Cases were re-examined by a dedicated head and neck pathologist; chart reviews supplemented data.
Main Results:
- 100% survival at 10 years was observed with CFR and negative surgical margins.
- Positive margins were associated with significantly poorer outcomes (survival <44 months).
- Advanced disease (tumor size ≥40 mm, Kadish C/D, TNM IVa/IVb) and positive margins predicted dismal survival.
Conclusions:
- Craniofacial resection (CFR), with or without adjuvant radiotherapy (XRT), is a safe and effective treatment for ONB.
- Achieving negative surgical margins is a critical factor for long-term survival.
- Negative margins, smaller tumor size (<40 mm), and earlier stages (Kadish A/B, TNM I-III) are independent predictors of favorable outcomes.
