Clear cell odontogenic carcinoma. A review

Area of Science:

• Oral Pathology
• Oncology
• Genetics

Background:

• Clear cell odontogenic carcinoma (CCOC) is a rare, difficult-to-diagnose malignant tumor.
• This review aims to demonstrate that CCOC is not exceptionally rare and to improve diagnostic strategies.

Purpose of the Study:

• To re-evaluate the rarity of CCOC.
• To compile and discuss the epidemiological, clinical, radiographic, histological, immunohistochemical, cytogenetic, management, follow-up, and prognostic features of CCOC.
• To enhance the diagnostic accuracy of CCOC.

Main Methods:

• A comprehensive literature search was conducted in Scopus, ScienceDirect, PubMed, and Medline databases (1985–2016).
• Data extraction focused on various aspects of CCOC, including epidemiology, clinical presentation, imaging, pathology, genetics, treatment, and outcomes.
• Ninety-five case reports from 65 studies were included.

Main Results:

• CCOC typically affects individuals in their fifth decade, most commonly in the mandible.
• Swelling, tooth mobility, and pain are frequent symptoms; radiologically, lesions appear radiolucent, mimicking cysts or periodontal issues.
• EWSR1 gene fission was frequently observed, and treatment often involved radical surgery with potential adjuvant therapies, though high recurrence and mortality rates were noted.

Conclusions:

• EWSR1 gene fission may be a crucial diagnostic marker for CCOC.
• A multidisciplinary approach involving radiologists, pathologists, and oral & maxillofacial surgeons is recommended for optimal management.
• The 95 reported cases suggest CCOC is more common than previously believed.