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Updated: Aug 19, 2025
Identification of OTX1 and OTX2 As Two Possible Molecular Markers for Sinonasal Carcinomas and Olfactory Neuroblastomas
Published on: February 28, 2019
Olfactory Neuroblastoma: Morphological Reappraisal and Molecular Insights with Quantum Leap in Clinical Perspectives
Diana Bell1, Andrew I Brandea2, Ehab Y Hanna3
1Anatomic Pathology and Head and Neck Disease Team Alignment, City of Hope Cancer Center, Duarte, CA, 91010, USA. dbell@coh.org.
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. 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.
Insights
Recent advances in olfactory neuroblastoma (ONB) diagnosis and treatment are highlighted. Molecular fingerprinting and targeted therapies show promise for improving outcomes in this rare cancer.
Area of Science:
- Oncology
- Molecular Biology
- Genetics
Background:
- Olfactory neuroblastoma (ONB) is a rare tumor requiring updated diagnostic and therapeutic strategies.
- Molecular alterations are increasingly recognized as crucial for understanding ONB pathogenesis and guiding treatment.
- Current diagnostic and treatment paradigms for ONB necessitate refinement based on emerging molecular data.
Purpose of the Study:
- To provide a comprehensive review of recent literature on olfactory neuroblastoma (ONB).
- To focus on advances in the diagnosis, molecular underpinnings, and targeted therapy of ONB.
- To explore the clinical implications of novel molecular findings in ONB.
Main Methods:
- Literature review of studies on olfactory neuroblastoma (ONB).
- Analysis of research focusing on molecular fingerprinting, including DNA methylation and IDH2 sequencing.
- Evaluation of studies investigating somatostatin receptor (SSTR) expression and its diagnostic/therapeutic potential.
Main Results:
- Molecular-based subtype classifications (basal-like ONB, neural-like ONB) are proposed but not widely adopted.
- DNA methylation analysis and IDH2 sequencing are gaining traction for routine ONB work-up.
- Somatostatin receptor (SSTR) expression offers new diagnostic avenues, especially for metastatic disease, and potential therapeutic targets with somatostatin analogs.
Conclusions:
- Molecular characterization of ONB is critical for developing novel treatment strategies.
- Further refinement of molecular biomarkers is needed for their integration into the clinical standard of care for ONB.
- Multi-institutional collaborative studies are essential for advancing the understanding and treatment of olfactory neuroblastoma.
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