REFCOR guidelines on the management of olfactory neuroblastoma: A formalized expert consensus

Olfactory neuroblastoma (ONB) is a rare malignant tumor of the sinonasal tract. The objective of this study is to present an evidence-based synthesis of the recommendations of the French Expert Network of Rare ENT Cancers (REFCOR), published in 2022, concerning the diagnostic and therapeutic management of ONB.

These recommendations result from a multidisciplinary consensus based on a critical analysis of the literature and feedback from expert centers within a structured process.

ONB accounts for 6% of sinonasal cancers, with no identified risk factors. Diagnosis is based on CT and MRI of the paranasal sinuses and skull base, together with systemic disease staging. Histopathological analysis requires specialized expertise, with Hyams histological grade as the main prognostic factor. The standard of care consists in surgical resection followed by adjuvant radiotherapy. Chemotherapy may be considered in high-grade or unresectable forms. Because of the risk of late recurrence, at least 20years' follow-up is recommended.

The REFCOR guidelines aim to standardize diagnosis and treatment for ONB, but require completion by discussion in a specialized multidisciplinary tumor board.

Published by European annals of otorhinolaryngology, head and neck diseases, 2026. The guideline itself is at the source linked here.

doi.org/10.1016/j.anorl.2026.06.004