NCI Definition: A rare neoplasm arising from tooth-forming tissues. It occurs in the maxillofacial skeleton or the gingiva. Symptoms include swelling, pain, bleeding, mobility of affected teeth, and oral mucosa ulcerations. It may metastasize to lymph nodes and distant anatomic sites early. [1]

Malignant odontogenic neoplasms most frequently harbor alterations in EWSR1, ATF1, SYNE1, RICTOR, and PRKDC [2].

Most Commonly Altered Genes in Malignant Odontogenic Neoplasm

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

Top Alterations in Malignant Odontogenic Neoplasm

Significant Genes in Malignant Odontogenic Neoplasm

CD274 +

Disease Details

Malignant Odontogenic Tumor, Odontogenic Carcinosarcoma, Odontogenic Carcinoma, Head and neck odontogenic carcinoma, Odontoma, Malignant
Odontogenic Neoplasm
Clear Cell Odontogenic Carcinoma
OncoTree Name
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.