Clear Cell Odontogenic Carcinoma (CCOC)

Overview

Clear Cell Odontogenic Carcinoma is an extremely rare malignant odontogenic tumor characterized by clear cells. It sits under the ODGC (Odontogenic Carcinoma) branch in OncoTree. CCOC has historically been poorly characterized molecularly; MSK-IMPACT profiling identified a novel EWSR1-ATF1 fusion not previously reported in odontogenic tumors.

Cohorts in the corpus

• hnc_mskcc_2016 — 1 CCOC case among 2 odontogenic carcinomas in 151 recurrent/metastatic head and neck tumors profiled by MSK-IMPACT 410-gene panel PMID:27442865.

Recurrent alterations

• EWSR1-ATF1 fusion — identified by MSK-IMPACT in the clear cell odontogenic carcinoma case; this EWSR1-ATF1 fusion had not been previously reported in odontogenic tumors; EWSR1-ATF1 is classically associated with clear cell sarcoma and angiomatoid fibrous histiocytoma PMID:27442865.

Subtypes

• Not further subclassified in the corpus.

Therapeutic landscape

• Therapeutic implications of the EWSR1-ATF1 fusion in CCOC are undefined; the finding nomads EWSR1-directed surveillance in all clear cell histology odontogenic tumors PMID:27442865.

Sources

• PMID:27442865 — Morris et al. 2017 (JAMA Oncol). Novel EWSR1-ATF1 fusion identified by MSK-IMPACT in 1 CCOC case; first report of this fusion in odontogenic tumors.

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