Int J Oral Biol.  2019 Sep;44(3):77-80. 10.11620/IJOB.2019.44.3.77.

Clear cell odontogenic carcinoma: a mini review

• Kim YH
• Seo EJ
• Park JK
• Jang IH

• KMID: 2460802
• DOI: http://doi.org/10.11620/IJOB.2019.44.3.77

Abstract

Clear cell odontogenic carcinoma (CCOC), a very rare neoplasm located mostly in the mandible, has been regarded as a benign tumor. However, due to the accumulation of case reports, CCOC has been reclassified as a malignant entity by the World Health Organization. Patients with CCOC present with regional swelling and periodontal indications with variable pain, often remaining misdiagnosed for a long period. CCOC has slow growth but aggressive behavior, requiring radical resection. Histologic analysis revealed the monophasic, biphasic, and ameloblastic types of CCOC with clear cells and a mixed combination of polygonal and palisading cells. At the molecular level, CCOC shows the expression of cytokeratin and epithelial membrane antigen, along with markers that assign CCOC to the sarcoma family. At the genetic level, Ewing sarcoma breakpoint region 1-activating transcription factor 1 fusion is regarded as the key feature for identification. Nevertheless, the scarcity of cases and dependence on histological data delay the development of an efficient therapy. Regarding the high recurrence rate and the potential of distant metastasis, further characterization of CCOC is necessary for an early and accurate diagnosis.

Keyword

Odontogenic tumor; Carcinoma; Clear cell

MeSH Terms

Ameloblasts
Diagnosis
Humans
Keratins
Mandible
Mucin-1
Neoplasm Metastasis
Odontogenic Tumors
Recurrence
Sarcoma
Sarcoma, Ewing
Transcription Factors
World Health Organization
Keratins
Mucin-1
Transcription Factors