ReviewCancer management and research2026
Systemic Therapy for High-Grade Olfactory Neuroblastoma: From Molecular Lineages to Targeted and Immune Strategies.
Review in Cancer management and research, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Authors and funding
6 authors.
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Abstract
Advanced olfactory neuroblastoma (ONB) remains a therapeutic challenge because evidence for systemic treatment is derived largely from small retrospective series and extrapolation from related neuroendocrine malignancies. This narrative review brings together diagnostic pathology, multi-omics classification, contemporary guideline-based management, and emerging systemic approaches for high-grade, recurrent, or metastatic ONB. Neural and Basal molecular programs provide a useful biological framework, but they are not validated tools for treatment selection. Delta-like ligand 3 (DLL3)-directed therapy and epigenetic-immune combinations are supported mainly by lineage-based or preclinical evidence, whereas somatostatin receptor (SSTR)-directed strategies and immune checkpoint blockade have generated only limited ONB-specific clinical data. All remain investigational. The first prospective immunotherapy trial in recurrent or metastatic ONB did not meet its objective-response endpoint, although prolonged disease stabilization was observed in a subset of patients. We therefore outline a practical approach that preserves established multidisciplinary care while using molecular and immune profiling to clarify diagnosis and guide clinical-trial referral. Routine biomarker-directed systemic therapy will require prospective multicenter validation using standardized assays and predefined biomarkers.
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