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Olfactory Neuroblastoma: Treatment Strategies for Advanced Disease

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Abstract

Purpose of Review

This review seeks to synthesize emerging literature and expert management recommendations of olfactory neuroblastoma (ONB), a rare sinonasal neuroendocrine malignancy.

Recent Findings

The rare nature of ONB and variability in extent of disease at presentation make formal recommendations and trials challenging—particularly as it pertains to advanced disease. Margin-negative surgical resection, including craniofacial and/or endoscopic approaches, when feasible, followed by radiation treatment performed with a multi-disciplinary care team remains the standard of care, with some evidence for chemotherapy in advanced disease. Salvage surgery and radiation for nodal metastases may provide extended periods of recurrence-free survival.

Summary

Management of ONB should include nuanced radiographic and anatomic staging followed by aggressive margin-negative surgical intervention coupled with radiation and possibly chemotherapy. Ongoing surveillance should be undertaken, even in patients with presumed definitive treatment due to high risk of delayed recurrence.

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Abbreviations

ONB:

Olfactory neuroblastoma

MIBG:

Metaiodobenzylguanidine

SEER:

Surveillance, Epidemiology, and End Results

IMRT:

Intensity-modulated radiation therapy

CSF:

Cerebrospinal fluid

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Authors and Affiliations

  1. Department of Neurologic Surgery, Mayo Clinic, 200 First St SW, Rochester, MN, 55905, USA

    Lucas P. Carlstrom & Jamie J. Van Gompel

  2. Department of Otorhinolaryngology, Mayo Clinic, 200 First St SW, Rochester, MN, 55905, USA

    Jamie J. Van Gompel & Garret Choby

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  1. Lucas P. Carlstrom
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Carlstrom, L.P., Van Gompel, J.J. & Choby, G. Olfactory Neuroblastoma: Treatment Strategies for Advanced Disease. Curr Otorhinolaryngol Rep 11, 117–127 (2023). https://doi.org/10.1007/s40136-023-00447-4

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