Atypical Presentation of Clear Cell Odontogenic Carcinoma
ABSTRACT Clear cell odontogenic carcinoma (CCOC) is a rare malignant neoplasm of odontogenic origin. The usual clinical presentation of CCOC is a mass of progressive growth in the mandible sometimes accompanied with loss of teeth, pain, or bleeding. We describe a rare case of CCOC that showed an atypical presentation not previously described in the literature like a fast-growing painless mass in the retromolar area that reached a considerable size in a few days that caused obstruction of the airway. The presence of airway obstruction required immediate treatment, which consisted of a surgical excision of the tumor via a hemimandibulectomy. This clinical report highlights the possibility of odontogenic tumors presenting like a rapid-growing mass and the importance of clinical differential diagnosis of such presentation.
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ABSTRACT: Clear cell odontogenic carcinoma (CCOC) is a rare odontogenic tumor of the jaws that is more common in the mandible than maxilla and has a female preponderance with a peak incidence in the sixth decade. It is characterized by locally aggressive behavior and has the potential to metastasize. This tumor was recently reported to have a rearrangement of the Ewing sarcoma breakpoint region one gene (EWSR1) in five of eight cases tested and the activating transcription factor one gene (ATF1) in one case tested. We report a case of CCOC in the premolar area of the mandible in a 59 year-old female. This case demonstrates the presence of both EWSR1 and ATF1 gene rearrangements by fluorescence in-situ hybridization (FISH).Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontology 10/2014; 118(4). DOI:10.1016/j.oooo.2014.02.004 · 1.46 Impact Factor