Atypical Presentation of Clear Cell Odontogenic Carcinoma

From the Departments of *Oral and Maxillofacial Surgery and †Pathology, Virgen del Rocio University Hospital, Seville, Spain.
The Journal of craniofacial surgery (Impact Factor: 0.68). 09/2012; 23(5):e466-8. DOI: 10.1097/SCS.0b013e31825757d4
Source: PubMed


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
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    ABSTRACT: The goal of this study is to describe 7 cases of clear cell odontogenic carcinoma among a Brazilian population and compare these data with a systematic review of the English-language literature. Descriptive statististics were used to compare the clinicopathologic data gathered retrospectively with those compiled from a review. Tumor sections were immunostained for Ki-67, p16, p53, and cytokeratins (CKs) 7, 8, 14, 18, and 19. Log-rank tests were performed for survival analysis. Most cases occurred in the posterior mandible (5/7, 71.4%), and recurrence was diagnosed in all treated patients. Metastatic disease occurred in 2 patients (28.6%). Tumors were focally positive for CKs 7, 8, 14, and 18 and diffusely positive for CK19, p53, and p16. The mean number of Ki-67-positive cells was 35.2 cells/high-power field. Our systematic review provided evidence that tumor size (P = .046), histologic pattern (P = .034), regional metastasis (P = .001), distant metastasis (P = .001), and local recurrence (P = .05) were of significant prognostic value. This study has contributed to improved characterization of clear cell odontogenic carcinoma, which is an aggressive odontogenic malignant neoplasm diagnosed mainly in the posterior mandible of middle-aged women and typically at an advanced stage. Radical surgical procedures remain the gold standard treatment. Copyright © 2015 Elsevier Inc. All rights reserved.
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    ABSTRACT: Background: Clear cell odontogenic carcinoma (CCOC) is a rare intraosseous carcinoma of the jaw; only 81 cases have been reported in the English literatures. Case presentation: We reported an additional case and reviewed the existing literature. A 70-year-old woman presented with a large painful radiolucent mandibular lesion from the right canine to the left angle area through the midline. No metastatic lymph nodes or distant metastases were detected. She underwent wide surgical resection and reconstruction with a composite fibula free flap. She had no recurrence or metastasis after 18 months. Conclusion: CCOC occurs predominantly in women in their 50s-70s in the mandible. Painless swelling is the most common symptom, followed by pain, teeth loosening, and paresthesia. CCOC has a good prognosis after surgery. In large mandibular CCOC, wide resection and composite fibula free flap reconstruction is the treatment of choice.
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