ArticleHead and neck pathology2021
Clear Cell Odontogenic Carcinoma: First Report of Novel EWSR1-CREM Fusion Gene in Case of Long-Term Misdiagnosis.
Article in Head and neck pathology, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 9 papers.
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9 citing papers in PubMed, 21 citations in OpenAlex.
- Integrating genotype-phenotype correlations into the diagnostic workflow of odontogenic tumors and cysts: insights from a 69 lesion cohort.Clinical oral investigations · 2025Article
- Diagnostic Dilemma of Clear Cell Odontogenic Carcinoma Mimicking Various Pathological Entities: A Case Report and Literature Review.Clinical case reports · 2025Article
- A case report and literature review: an intra-abdominal epithelioid neoplasm with EWSR1::CREB fusions originating from the kidney.Frontiers in oncology · 2025Article
- Loss of clear cell characteristics in aggressive clear cell odontogenic carcinoma: a case report.Diagnostic pathology · 2024Article
- Diagnostic Utility of EWSR1 in Clear Cell Odontogenic Carcinoma: A Systematic Review.Indian journal of otolaryngology and head and neck surgery : official publication of the Association of Otolaryngologists of India · 2024Article
- Article
- Clear cell odontogenic carcinoma of jawbone diagnosed by fluorescence in situ testing in Ewing sarcoma RNA binding protein 1 gene rearrangements: A rare case report.Journal of dental sciences · 2023Article
- Metastatic sporadic paraganglioma with EWSR1::CREM gene fusion: A unique molecular profile that expands the phenotypic diversity of the molecular landscape of the EWSR1::CREM gene fusion positive tumors.Genes, chromosomes & cancer · 2023Article
- Comprehensive genomic profiling of EWSR1/FUS::CREB translocation-associated tumors uncovers prognostically significant recurrent genetic alterations and methylation-transcriptional correlates.Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc · 2022Article
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Authors and funding
6 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Clear Cell odontogenic Carcinomas (CCOC) are rare, aggressive malignant odontogenic tumours which are often misdiagnosed as benign odontogenic tumours due to the non-specific histologic appearance, and benign early clinical presentation. However, due to their propensity to metastasize, the best outcomes are experienced with they are diagnosed early and treated aggressively. In this paper, we present a case of a CCOC misdiagnosed as a clear cell calcifying epithelial odontogenic tumour which was only found to be a CCOC after cervical node metastasis. The original diagnosis was questioned and confirmed to be a CCOC by identification of the chromosomal translocation EWSR1 on fluorescence in situ hybridization. This has recently been described in CCOC and a wide variety of other mesenchymal and epithelial neoplasms. Previous reports have demonstrated EWSR1-ATF1 and EWSR1-CREB1 fusions in CCOC. Next generation sequencing of this case demonstrated the EWSR1-CREM fusion gene which has not been previously reported for CCOC. CREM fusion proteins have only recently been found in several tumour types including the closely associated hyalinizing clear cell carcinoma of salivary glands. This is discussed in this paper, and the role of the discovery of the CREM fusion protein in CCOC adds to your understating of the role of CREM in oncogenesis, and the possible link between CCOCs and hyalinizing clear cell carcinomas.
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