NCI Definition: A usually aggressive malignant neoplasm arising from tooth-forming tissues. It more often affects older females and more frequently occurs in the mandible. It is characterized by the presence of malignant epithelial cells with clear cytoplasm and a fibrotic stroma formation. It may recur and metastasize. Metastases may occur in the lymph nodes, lungs, and bones. Treatment of choice is resection with clean margins. [1]

Clear cell odontogenic carcinomas most frequently harbor alterations in EWSR1, ATF1, SYNE1, RICTOR, and PRKDC [2].

Most Commonly Altered Genes in Clear Cell Odontogenic Carcinoma

EWSR1-ATF1 Fusion, EWSR1 Fusion, SYNE1 R5410Q, RICTOR Mutation, and RICTOR L212F are the most common alterations in clear cell odontogenic carcinoma [2].

Top Alterations in Clear Cell Odontogenic Carcinoma

Disease Details

Clear Cell Ameloblastoma, Clear Cell Odontogenic Tumor
Malignant Odontogenic Neoplasm
OncoTree Name
Clear Cell Odontogenic Carcinoma
OncoTree Code


1. National Cancer Institute. NCI Thesaurus Version 18.11d. https://ncit.nci.nih.gov/ncitbrowser/ [2018-08-28]. [2018-09-21].

2. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 8. This dataset does not represent the totality of the genetic landscape; see paper for more information.

3. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.