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Purpose of review Olfactory neuroblastoma (ONB) is a rare malignancy originating from olfactory neuroepithelial cells. Given its uncommon nature and complex clinical presentation, this comprehensive review highlights recent findings and treatment approaches for advancing clinical practice and research. Recent findings Recent literature emphasizes significant advancements in the genomic profiling and molecular classification of ONB. Emerging targeted therapies include somatostatin analogs and programmed death-ligand 1 (PD-L1) inhibitors. In addition, the development of genetically engineered mouse models has provided valuable platforms for testing new treatment strategies, revealing similarities between ONB and small cell lung cancer, which may inform future therapeutic approaches. Summary These findings have profound implications on clinical practice. Improved diagnostic accuracy through advanced imaging and genomic profiling in addition to identifying specific mutations for targeted therapy can lead to personalized treatments of patients with ONB. Developments in genetically engineered mouse models and multiinstitutional collaborative efforts are vital for advancing research and standardizing molecular testing. The integration of advanced imaging techniques, genomic profiling, and targeted therapies holds promise for improving patient outcomes and understanding this rare malignancy.
Purpose of review Olfactory neuroblastoma (ONB) is a rare malignancy originating from olfactory neuroepithelial cells. Given its uncommon nature and complex clinical presentation, this comprehensive review highlights recent findings and treatment approaches for advancing clinical practice and research. Recent findings Recent literature emphasizes significant advancements in the genomic profiling and molecular classification of ONB. Emerging targeted therapies include somatostatin analogs and programmed death-ligand 1 (PD-L1) inhibitors. In addition, the development of genetically engineered mouse models has provided valuable platforms for testing new treatment strategies, revealing similarities between ONB and small cell lung cancer, which may inform future therapeutic approaches. Summary These findings have profound implications on clinical practice. Improved diagnostic accuracy through advanced imaging and genomic profiling in addition to identifying specific mutations for targeted therapy can lead to personalized treatments of patients with ONB. Developments in genetically engineered mouse models and multiinstitutional collaborative efforts are vital for advancing research and standardizing molecular testing. The integration of advanced imaging techniques, genomic profiling, and targeted therapies holds promise for improving patient outcomes and understanding this rare malignancy.
Introduction: Olfactory carcinoma (OC) is a rare tumor that is often misdiagnosed as olfactory neuroblastoma (ONB). Due to its rarity, there is no established standard treatment for OC, and there have been no reports of successful long-term follow-up treatment yet. Case Presentation: Two male patients, aged 54 and 48, presented with similar symptoms of nasal obstruction, anosmia, facial swelling, and epistaxis. Imaging in both cases revealed large, locally advanced sinonasal neoplasms. Initial biopsies were concerning for ONB, however final pathological analyses confirmed high grade OC. Treatment comprised induction chemotherapy, extensive skull base tumor resection, adjuvant chemoradiotherapy, and reconstructive surgery in both cases. The 54-year-old remains disease free at 7 years, while the 48-year-old demonstrates no evidence of disease at 8 months. Conclusion: Accurate diagnostic differentiation is imperative to optimize a treatment approach. Although outcomes with OC tend to be worse than those of ONB, effective control can be achieved with judicious concurrent use of neoadjuvant, surgery, and adjuvant treatments. Ongoing surveillance, biobanking, and genomic analysis are crucial to gain a better understanding of this complex disease.
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