Clear Cell Odontogenic Neoplasms
Summary
Clear cell odontogenic neoplasms are a spectrum of rare jaw tumours arising from odontogenic epithelium, distinguished by nests or sheets of cells with clear cytoplasm due to intracellular glycogen. They range from benign but locally aggressive entities to malignant clear cell odontogenic carcinoma (CCOC), which was formally recognised in the WHO classification of head and neck tumours. These neoplasms predominantly affect middle-aged adults, exhibit a slight female predilection and often involve the mandible. Clinically they present with painless or painful swelling, tooth mobility, paresthesia or radiological findings of poorly defined radiolucencies. Histopathological hallmarks include uniform clear cells in a hyalinised stroma, variable cellular atypia, mitotic figures and infiltrative growth patterns. Molecular insights, notably recurrent EWSR1 gene fusions, have enhanced diagnostic precision. Management centres on wide surgical resection with clear margins, appropriate reconstruction and long-term surveillance to address risks of local recurrence and rare distant metastasis.
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Clear Cell Odontogenic Neoplasms publication trend
The graph below shows the total number of articles in clear cell odontogenic neoplasms across all publications each year (not limited to Nature Index journals).
Technical terms
Clear cell: Epithelial cell with pale cytoplasm due to glycogen or lipid content, appearing optically clear under light microscopy.
EWSR1 rearrangement: A genetic fusion involving the EWSR1 gene commonly found in certain sarcomas and odontogenic carcinomas, used as a molecular diagnostic marker.
Perineural invasion: Tumour infiltration along nerve sheaths, associated with aggressive behaviour and increased recurrence risk.
Immunohistochemistry: Laboratory technique using labelled antibodies to detect specific proteins in tissue sections, aiding in tumour classification.
Hyalinised stroma: Dense, glassy collagenous background between tumour cells, characteristic of certain neoplasms.
References
- Unusual Presentation of Clear Cell Odontogenic Carcinoma: Case Report and Literature Review. Diagnostics (2022).
- Loss of clear cell characteristics in aggressive clear cell odontogenic carcinoma: a case report. Diagnostic Pathology (2024).
- Clear Cell Odontogenic Carcinoma: A Series of Three Cases. Dentistry Journal (2022).
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