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Olfactory neuroblastoma: a single-center experience

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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.

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Authors and Affiliations

  1. Department of Neurosurgery, Oslo University Hospital—Rikshospitalet, PB4950 Nydalen, 0424, Oslo, Norway

    Marton König & Torstein R. Meling

  2. Institute of Clinical Medicine, Faculty of Medicine, University of Oslo, Oslo, Norway

    Marton König, Terje Osnes & Torstein R. Meling

  3. Department of Otorhinolaryngology, Oslo University Hospital—Rikshospitalet, PB4950 Nydalen, 0424, Oslo, Norway

    Terje Osnes

  4. Department of Pathology, Oslo University Hospital—Rikshospitalet, PB4950 Nydalen, 0424, Oslo, Norway

    Peter Jebsen

  5. Department of Oncology, Oslo University Hospital—Radiumhospitalet, PB4950 Nydalen, 0424, Oslo, Norway

    Jan Folkvard Evensen

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  1. Marton König
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Correspondence to Marton König.

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This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

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This study was approved by the data protection official at OUH (ePhorte 2015-5042). All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. This study does not contain any studies with animals performed by any of the authors.

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König, M., Osnes, T., Jebsen, P. et al. Olfactory neuroblastoma: a single-center experience. Neurosurg Rev 41, 323–331 (2018). https://doi.org/10.1007/s10143-017-0859-3

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