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Update on olfactory neuroblastoma

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Abstract

Olfactory neuroblastomas are uncommon malignancies that arise from olfactory receptor cells located high in the nasal cavity. Accurate diagnosis plays a crucial role in determining clinical results and guiding treatment decisions. Diagnosis can be a major challenge for pathologists, especially when dealing with tumours with poor differentiation. The discovery of several molecular and immunohistochemical markers would help to overcome classification difficulties. Due to the paucity of large-scale studies, standardisation of diagnosis, treatment and prediction of outcome remains a challenge. Surgical resection by endoscopic techniques with the addition of postoperative irradiation is the treatment of choice. In addition, it is advisable to consider elective neck irradiation to minimise the risk of nodal recurrence. Molecular characterisation will help not only to make more accurate diagnoses but also to identify specific molecular targets that can be used to develop personalised treatment options tailored to each patient. The present review aims to summarise the current state of knowledge on histopathological diagnosis, the molecular biology and management of this disease.

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Fernando López, Abbas Agaimy and Alessandro Franchi contributed to the writing of manuscript; Fernando López and Alfio Ferlito contributed to conceptualisation; Abbas Agaimy and Alessandro Franchi contributed to the production of the figures; Carlos Suárez, Vincent Vander Poorten, Antti A. Mäkitie, Akihiro Homma, Avraham Eisbruch, Kerry D. Olsen, Nabil F Saba, Sandra Nuyts, Carl Snyderman, Jonathan J. Beitler, June Corry, Ehab Hanna, Henrik Hellquist, Alessandra Rinaldo and Alfio Ferlito critically revised the work and Abbas Agaimy and Alfio Ferlito approved the final version to be published.

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Correspondence to Fernando Lopez.

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Lopez, F., Agaimy, A., Franchi, A. et al. Update on olfactory neuroblastoma. Virchows Arch 484, 567–585 (2024). https://doi.org/10.1007/s00428-024-03758-z

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