Other sinonasal tumors
2025-04-08 Paola Dal Cin, PhD Affiliation1.Brigham and Women's Hospital , Harvard Medical School, Boston , MA (USA)
Keywords
Neuropathologic sinonasal tract entities, olfactory neuroblastomaClassification
Definition
Neuropathologic entities uncommonly involve the sinonasal tract and intracranial tumors of the skull base can involve structures of the head and neck by direct extension or rarely metastasis. 1
There is limited information about genomic make up olfactory neuroblastoma (ONB). Few genome studies are controversial from a high degree of chromosomal instability and gene gains, to chromosomal stability and a predominance of gene losses. 2 Molecular-based subtype ONB classifications have been proposed but are not currently in wide use. 3,4
| Other sinonasal tumors | Genetic marker(s) |
|---|---|
| Sinonasal ameloblastoma | Sinonasal ameloblastomas are histologically similar to gnathic ameloblastomas, lacking both lack human papillomavirus DNA,5 but with different clinical presentation .6 The presence of BRAF mutation has not be yet tested in sinomonasal ameloblastomas. |
| Sinonasal adamantinomatous craniopharyngioma (ACP) | It is usual located in a sellar and supersellar regions, arising most often in children, but rare cases have been reported in sinonasal region.1 The presence of CTNNB1 mutation has not be yet tested in sinonasal NDUFAB1. |
| Meningioma of the sinonasal tract, ear, and temporal bone | Rarely primary extracranial meningiomas can occur in the head and neck , including nasal cavity and paranasal sinus.1,7 A number of genetic and molecular pathways are disrupted in meningioma, their presence have not be yet tested in sinonasal areas,however, Grade II and III tumors or with tumor progression, features not normally present in the sinonasal tract.8 |
| Olfactory neuroblastoma (ONB) | Olfactory neuroblastoma (ONB) a.k.a. esthesioneuroblastoma,is a rare malignancy originating from olfactory neuroepithelial cells, typically occuring in the superior nasal cavity, and it is most common in adults in the fifth to sixth decades of life.2 Chromosome changes in ONB have been studied using different techniques, and complex karyotypes with heterogeneous chromosomal instability, variering widely among ONB tumors, generally without balanced translocations have been reported. Loss of 3p was found to be the most common chromosomal alteration reported to be associated with resistance to chemotherapy or radiotherapy , chromosome 11 deletion and chromosome 1p gain have been associated with poor ONB survival, and alterations in 20q and 13q have been more frequently associated with advanced-stage tumors. 9 |
| Extensive sequecings confirmed that ONB is genetically a heterogeneous entity, with more frequent mutations in the metastasis than in primary tumors. The most frequent altered gene was TP53, following by PIK3CA, NF1, CDKN2A or CDKN2C, 10-12 and FGFR3 and CCND1 copy-number alterations or amplifications are reported. 13 IDH2 mutations represented a distinct subset of ONB with aggressive behavior represent an independent poor prognostic factor and could be used as a therapeutic target. 14 An unexpected possibe functional role for DMD and LAMA2 causing hereditary muscular dystrophies in ONB have been suggested. 15 |
Article Bibliography
| Reference Number | Pubmed ID | Last Year | Title | Authors |
|---|---|---|---|---|
| 1 | 39489560 | 2024 | Neuropathology Entities Involving the Sinonasal Tract. | Roberts KF et al |
| 2 | 38386106 | 2024 | Update on olfactory neuroblastoma. | Lopez F et al |
| 3 | 29730775 | 2018 | DNA methylation-based reclassification of olfactory neuroblastoma. | Capper D et al |
| 4 | 30332658 | 2018 | Integrated Multi-omic Analysis of Esthesioneuroblastomas Identifies Two Subgroups Linked to Cell Ontogeny. | Classe M et al |
| 5 | 26190154 | 2015 | Gnathic and peripheral ameloblastomas lack human papillomavirus DNA. | Verduin L et al |
| 6 | 36615180 | 2023 | Clinicopathologic Profile, Management and Outcome of Sinonasal Ameloblastoma-A Systematic Review. | Mehta V et al |
| 7 | 24427682 | 2013 | Primary extracranial meningioma of paranasal sinuses. | Aiyer RG et al |
| 8 | 26830398 | 2016 | Update on Select Benign Mesenchymal and Meningothelial Sinonasal Tract Lesions. | Thompson LDR et al |
| 9 | 36449116 | 2023 | Olfactory Neuroblastoma: Morphological Reappraisal and Molecular Insights with Quantum Leap in Clinical Perspectives. | Bell D et al |
| 10 | 27256979 | 2016 | Genetic and molecular alterations in olfactory neuroblastoma: implications for pathogenesis, prognosis and treatment. | Czapiewski P et al |
| 11 | 28495808 | 2017 | Comprehensive Genomic Profiling of Esthesioneuroblastoma Reveals Additional Treatment Options. | Gay LM et al |
| 12 | 29324814 | 2018 | Comprehensive molecular profiling of advanced/metastatic olfactory neuroblastomas. | Topcagic J et al |
| 13 | 28775129 | 2017 | Comprehensive Molecular Profiling of Olfactory Neuroblastoma Identifies Potentially Targetable FGFR3 Amplifications. | Lazo de la Vega L et al |
| 14 | 34856401 | 2022 | Clinicopathologic Features and Prognosis of Olfactory Neuroblastoma with Isocitrate Dehydrogenase 2 Mutations. | Wu L et al |
| 15 | 30575736 | 2018 | Genomic analysis identifies frequent deletions of Dystrophin in olfactory neuroblastoma. | Gallia GL et al |
Citation
Paola Dal Cin, PhD
Other sinonasal tumors
Atlas Genet Cytogenet Oncol Haematol. 2025-04-08
Online version: http://atlasgeneticsoncology.org/solid-tumor/209321/other-sinonasal-tumors
