Odontogenic Carcinoma with Dentinoid: A Case Report and Review of Literature

Abstract

Introduction

Odontogenic carcinoma with dentinoid (OCD) is a rare entity found in the head and neck region particularly involving the jaws. Histopathology is remarkable for its well-circumscribed lesion composed of epithelioid tumor cells with eosinophilic to clear cytoplasm admixed with a diffuse dentinoid matrix. Conventionally, focal dentinoid histomorphology is nonspecific and can be found in many benign odontogenic tumors such as odontoma, adenomatoid odontogenic tumor, and calcifying odontogenic cyst. However, diffuse dentinoid phenotype in OCD is one of the key diagnostic features. This overlapping morphology has often led to a misdiagnosis. OCD has shown pathogenic mutations in CTNNB1 and APC genes in the Wnt-pathway which is also found in other odontogenic tumors such as dentinogenic ghost cell tumor, dentinogenic ghost cell carcinoma, and adenoid ameloblastoma. Hence, further studies are needed to observe if harboring the same mutations demonstrate any similarities in disease behavior and prognosis.

Material and methods

A case of a 19-year-old male with a left nasal mass filling the entire maxillary sinus diagnosed as odontogenic carcinoma with dentinoid is presented. In addition, a search of the English-language literature retrieved additional cases of odontogenic carcinoma with dentinoid.

Results

Approximately 11 additional cases were found in the MEDLINE/PubMed literature search. Disease characteristics including epidemiological factors, clinical presentations, and histology are reviewed and data recorded.

Conclusion

Odontogenic carcinoma with dentinoid should be considered as a differential when a biopsy of a nodular lesion in the jaws shows dentinoid features. Clinical and radiologic correlation along with consideration for rebiopsy is recommended when suspicion arises.