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[115]
overall survival of 32 months in a setting of relatively mild toxicity profile . This study illustrates the
promising utility of targeted receptor therapy for metastatic non-resectable disease. While larger,
randomized trials are necessary to determine the efficacy of such targeted therapies over standard-of-care,
the idea of borrowing validated treatment approaches from other neuroendocrine cancers may represent a
promising path forward.

Finally, with the rapid rise in immunotherapies for solid tumors, there has been interest in the olfactory
neuroblastoma immune landscape. Classe et al. observed an increase in tumor infiltrating lymphocytes in
[112]
high grade compared to low-grade tumors . London et al. later demonstrated in a cohort of ten olfactory
neuroblastomas that 4/10 tumors were positive for the immune checkpoint Programmed death ligand 1
(PD-L1), whereas in metastatic samples, 3/4 tumors stained positive for PD-L1 . This prompted a phase 2
[116]
trial currently underway, assessing the role of Bintrafusp Alfa, a bifunctional fusion protein targeting PD-L1
and transforming growth factor (TGF)-beta signaling, in recurrent olfactory neuroblastoma . Depending
[117]
on the outcome, immune checkpoint inhibitor therapy could be a future option for some olfactory
neuroblastoma patients.

CONCLUSION
Olfactory neuroblastoma is a rare malignant tumor, originating from the olfactory neuroepithelium. Due to
the malignant nature of the tumor, proximity to the brain and orbit, and tendency for cervical lymph node
metastasis, a comprehensive clinical assessment should include detailed head and neck imaging, to properly
stage the tumor. A multidisciplinary approach involving head and neck surgeons, neurosurgeons, radiation,
and medical oncologists should be used for management planning. The surgical management of olfactory
neuroblastoma should aim for negative margins using either endoscopic endonasal, open or hybrid
approaches. Endoscopic endonasal approach often provides complete surgical resection with lower
morbidities compared to open approaches. Advanced tumor stage requires more aggressive treatment
strategies with combined surgical approaches and adjuvant or neoadjuvant therapies as olfactory
neuroblastoma has shown sensitivity to both chemotherapy and radiation therapy. Given the tumor’s
tendency for late recurrence, extended post-treatment surveillance remains necessary.

DECLARATIONS
Authors’ contributions
Conceived and designed the book chapter: Abi Hachem R
Coordinated various parts of the study: Okafor S, AlShammari S, Helou V, Mitchell M, Finlay JB
Had full access to all the data in the study and took responsibility for the integrity and accuracy of the study
analysis: Abi Hachem R, Goldstein B, Jang D
Wrote the article: Okafor S, AlShammari S, Helou V, Mitchell M, Finlay JB
Provided cyclical feedback on the writing process: Abi Hachem R, Goldstein B, Jang D
All authors have reviewed and approved the final manuscript.

Availability of data and materials
All data generated or analyzed during this study are included in this published article.

Financial support and sponsorship
None.

Conflicts of interest
All authors declared that there are no conflicts of interest.