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Olfactory Neuroblastoma: Morphological Reappraisal and Molecular Insights with Quantum Leap in Clinical Perspectives

  • Head and Neck Cancers (EY Hanna, Section Editor)
  • Published:
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Abstract

Purpose of Review

The purpose of review is to provide a comprehensive review of the literature focusing on the recent advances in the diagnosis, molecular underpinning, and targeted therapy of olfactory neuroblastoma (ONB).

Recent Findings

Studies focused on the molecular fingerprinting of ONB are critical to engage new promising treatment strategies. Molecular-based subtype classifications have been proposed (basal-like ONB and neural-like ONB) but are not widely used. The rationale for implementation of DNA methylation analysis and IDH2 sequencing in routine work-up for ONB is gaining recognition. Expression of somatostatin receptors (SSTR) in ONB open new avenues for both, diagnostic (especially metastatic disease) and new treatment protocols with somatostatin analogs. Olfactory carcinoma is proposed as a unifying diagnostic terminology pertinent to epithelial divergent differentiation in olfactory neuroblastoma.

Summary

Molecular (genetic and epigenetic) efforts on olfactory neuroblastoma are promising; however further refinement is needed for employment of these biomarkers as clinical standard of care. Ongoing and future multi-institutional collaborative studies will contribute to further understanding of ONB biology and aid the development of targeted treatments for this disease.

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Authors and Affiliations

  1. Anatomic Pathology and Head and Neck Disease Team Alignment, City of Hope Cancer Center, Duarte, CA, 91010, USA

    Diana Bell

  2. Nova Southeastern University, Fort Lauderdale, FL, 33314, USA

    Andrew I. Brandea

  3. Division of Head and Neck Surgery and Neurosurgery, MD Anderson Cancer Center, Houston, TX, 77030, USA

    Ehab Y. Hanna

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Bell, D., Brandea, A.I. & Hanna, E.Y. Olfactory Neuroblastoma: Morphological Reappraisal and Molecular Insights with Quantum Leap in Clinical Perspectives. Curr Oncol Rep 25, 11–18 (2023). https://doi.org/10.1007/s11912-022-01348-3

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